Human Metabolome Database Version 2.5

Browsing diseases

11-beta-Hydroxylase deficiency (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB04030	21-Deoxycortisol	Blood	Tonetto-Fernandes V, Lemos-Marini SH, Kuperman H, Ribeiro-Neto LM, Verreschi IT, Kater CE: Serum 21-Deoxycortisol, 17-Hydroxyprogesterone, and 11-deoxycortisol in classic congenital adrenal hyperplasia: clinical and hormonal correlations and identification of patients with 11beta-hydroxylase deficiency among a large group with alleged 21-hydroxylase deficiency. J Clin Endocrinol Metab. 2006 Jun;91(6):2179-84. Epub 2006 Mar 21. [PubMed ]

2-Aminoadipic aciduria (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00510	Aminoadipic acid	Blood; Urine	Metagene: 2-AMINOADIPIC ACIDURIA

2-Hydroxyglutaric Aciduria (D) (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00606	D-2-Hydroxyglutaric acid	Blood; CSF; Urine	Metagene: 2-HYDROXYGLUTARIC ACIDURIA (D)

2-Hydroxyglutaric acid Aciduria (D) (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00606	D-2-Hydroxyglutaric acid	Blood; CSF; Urine	Metagene: 2-HYDROXYGLUTARIC ACIDURIA (D)

2-Hydroxyglutaric Acidemia (L) (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00694	L-2-Hydroxyglutaric acid	Blood; CSF; Urine	Metagene: 2-HYDROXYGLUTARIC ACIDEMIA (L)

2-Hydroxyglutaric Aciduria (D) (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00606	D-2-Hydroxyglutaric acid	Blood; CSF; Urine	Metagene: 2-HYDROXYGLUTARIC ACIDURIA (D)

2-ketoadipic Acidemia (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00510	Aminoadipic acid	Blood; Urine	Metagene: 2-KETOADIPIC ACIDEMIA

2-Ketoadipic Acidemia (2 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00321	2-Hydroxyadipic acid	Urine	Metagene: 2-KETOADIPIC ACIDEMIA
HMDB00225	Oxoadipic acid	Urine	Metagene: 2-KETOADIPIC ACIDEMIA

2-Ketoglutarate Dehydrogenase Comples Deficiency (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00208	Oxoglutaric acid	Blood; Cellular Cytoplasm; CSF; N/A; Urine	Metagene: 2-KETOGLUTARATE DEHYDROGENASE COMPLEX%2

21-Hydroxylase deficiency (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB04030	21-Deoxycortisol	Blood	Tonetto-Fernandes V, Lemos-Marini SH, Kuperman H, Ribeiro-Neto LM, Verreschi IT, Kater CE: Serum 21-Deoxycortisol, 17-Hydroxyprogesterone, and 11-deoxycortisol in classic congenital adrenal hyperplasia: clinical and hormonal correlations and identification of patients with 11beta-hydroxylase deficiency among a large group with alleged 21-hydroxylase deficiency. J Clin Endocrinol Metab. 2006 Jun;91(6):2179-84. Epub 2006 Mar 21. [PubMed ]

21-Hydroxylase Deficiency (CYP21) (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00374	17-Hydroxyprogesterone	Blood; Saliva; Urine	Metagene: 21-HYDROXYLASE DEFICIENCY (CYP21)

3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency (7 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00754	3-Hydroxyisovaleric acid	Blood; CSF; Urine	Metagene: 3-HYDROXY-3-METHYLGLUTARYL-CoA LYASE DEFICIENCY
HMDB00355	3-Hydroxymethylglutaric acid	Urine	Metagene: 3-HYDROXY-3-METHYLGLUTARYL-CoA LYASE DEFICIENCY
HMDB00459	3-Methylcrotonylglycine	Urine	Metagene: 3-HYDROXY-3-METHYLGLUTARYL-CoA LYASE DEFICIENCY
HMDB00522	3-Methylglutaconic acid	Blood; Urine	Metagene: 3-HYDROXY-3-METHYLGLUTARYL-CoA LYASE DEFICIENCY
HMDB00448	Adipic acid	Blood; CSF; Urine	Metagene: 3-HYDROXY-3-METHYLGLUTARYL-CoA LYASE DEFICIENCY
HMDB00051	Ammonia	Blood; Cellular Cytoplasm; CSF; Urine	Metagene: 3-HYDROXY-3-METHYLGLUTARYL-CoA LYASE DEFICIENCY
HMDB00752	Methylglutaric acid	Urine	Metagene: 3-HYDROXY-3-METHYLGLUTARYL-CoA LYASE DEFICIENCY

3-Hydroxyisobutyric Aciduria (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00023	(S)-3-Hydroxyisobutyric acid	Blood; CSF; Urine	Metagene: 3-HYDROXYISOBUTYRIC ACIDURIA

3-Methyl-Crotonyl-Glycinuria (3 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00754	3-Hydroxyisovaleric acid	Blood; CSF; Urine	Metagene: 3-METHYL-CROTONYL-GLYCINURIA
HMDB00459	3-Methylcrotonylglycine	Urine	Metagene: 3-METHYL-CROTONYL-GLYCINURIA
HMDB00051	Ammonia	Blood; Cellular Cytoplasm; CSF; Urine	Metagene: 3-METHYL-CROTONYL-GLYCINURIA

3-Methylglutaconic aciduria (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00754	3-Hydroxyisovaleric acid	Blood; CSF; Urine	Engelke UF, Kremer B, Kluijtmans LA, van der Graaf M, Morava E, Loupatty FJ, Wanders RJ, Moskau D, Loss S, van den Bergh E, Wevers RA: NMR spectroscopic studies on the late onset form of 3-methylglutaconic aciduria type I and other defects in leucine metabolism. NMR Biomed. 2006 Apr;19(2):271-8. [PubMed ]

3-Methylglutaconic Aciduria (TYPE I) (3 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00754	3-Hydroxyisovaleric acid	Blood; CSF; Urine	Metagene: 3-METHYLGLUTACONIC ACIDURIA (TYPE I)
HMDB00522	3-Methylglutaconic acid	Blood; Urine	Metagene: 3-METHYLGLUTACONIC ACIDURIA (TYPE I)
HMDB00752	Methylglutaric acid	Urine	Metagene: 3-METHYLGLUTACONIC ACIDURIA (TYPE I)

3-Methylglutaconic Aciduria (TYPE II), X-LINKED (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00243	Pyruvic acid	Blood; Cellular Cytoplasm; CSF; Saliva; Urine	Metagene: 3-METHYLGLUTACONIC ACIDURIA (TYPE II), X-LINKED

4-Hydroxybutyric Aciduria (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00337	(S)-3,4-Dihydroxybutyric acid	Blood; CSF; Urine	Metagene: 4-HYDROXYBUTYRIC ACIDURIA

5-Oxoprolinase Deficiency (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00267	Pyroglutamic acid	Blood; CSF; Urine	Metagene: 5-OXOPROLINASE DEFICIENCY

Abetalipoproteinemia (9 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB04844	Ganglioside GM3 (d18:1/16:0)	Blood	Dawson G, Kruski AW, Scanu AM: Distribution of glycosphingolipids in the serum lipoproteins of normal human subjects and patients with hypo- and hyperlipidemias. J Lipid Res. 1976 Mar;17(2):125-31. [PubMed ]
HMDB00140	Glucosylceramide	Blood	Dawson G, Kruski AW, Scanu AM: Distribution of glycosphingolipids in the serum lipoproteins of normal human subjects and patients with hypo- and hyperlipidemias. J Lipid Res. 1976 Mar;17(2):125-31. [PubMed ]
HMDB04866	Lactosylceramide (d18:1/12:0)	Blood	Dawson G, Kruski AW, Scanu AM: Distribution of glycosphingolipids in the serum lipoproteins of normal human subjects and patients with hypo- and hyperlipidemias. J Lipid Res. 1976 Mar;17(2):125-31. [PubMed ]
HMDB04958	Tetrahexosylceramide (d18:1/12:0)	Blood	Dawson G, Kruski AW, Scanu AM: Distribution of glycosphingolipids in the serum lipoproteins of normal human subjects and patients with hypo- and hyperlipidemias. J Lipid Res. 1976 Mar;17(2):125-31. [PubMed ]
HMDB04877	Trihexosylceramide (d18:1/12:0)	Blood; Urine	Dawson G, Kruski AW, Scanu AM: Distribution of glycosphingolipids in the serum lipoproteins of normal human subjects and patients with hypo- and hyperlipidemias. J Lipid Res. 1976 Mar;17(2):125-31. [PubMed ]
HMDB04880	Trihexosylceramide (d18:1/18:0)	Blood; Urine	Dawson G, Kruski AW, Scanu AM: Distribution of glycosphingolipids in the serum lipoproteins of normal human subjects and patients with hypo- and hyperlipidemias. J Lipid Res. 1976 Mar;17(2):125-31. [PubMed ]
HMDB04882	Trihexosylceramide (d18:1/22:0)	Blood; Urine	Dawson G, Kruski AW, Scanu AM: Distribution of glycosphingolipids in the serum lipoproteins of normal human subjects and patients with hypo- and hyperlipidemias. J Lipid Res. 1976 Mar;17(2):125-31. [PubMed ]
HMDB04886	Trihexosylceramide (d18:1/24:0)	Blood; Urine	Dawson G, Kruski AW, Scanu AM: Distribution of glycosphingolipids in the serum lipoproteins of normal human subjects and patients with hypo- and hyperlipidemias. J Lipid Res. 1976 Mar;17(2):125-31. [PubMed ]
HMDB04884	Trihexosylceramide (d18:1/26:1(17Z))	Blood; Urine	Dawson G, Kruski AW, Scanu AM: Distribution of glycosphingolipids in the serum lipoproteins of normal human subjects and patients with hypo- and hyperlipidemias. J Lipid Res. 1976 Mar;17(2):125-31. [PubMed ]

Abnormal (46 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00338	2-Hydroxyestradiol	Urine	Reichman ME, Judd JT, Longcope C, Schatzkin A, Clevidence BA, Nair PP, Campbell WS, Taylor PR: Effects of alcohol consumption on plasma and urinary hormone concentrations in premenopausal women. J Natl Cancer Inst. 1993 May 5;85(9):722-7. [PubMed ]
HMDB00343	2-Hydroxyestrone	Blood; Urine	Reichman ME, Judd JT, Longcope C, Schatzkin A, Clevidence BA, Nair PP, Campbell WS, Taylor PR: Effects of alcohol consumption on plasma and urinary hormone concentrations in premenopausal women. J Natl Cancer Inst. 1993 May 5;85(9):722-7. [PubMed ]
HMDB05998	20-Hydroxyeicosatetraenoic acid	Blood; CSF; Urine	Barden A, Zilkens RR, Croft K, Mori T, Burke V, Beilin LJ, Puddey IB: A reduction in alcohol consumption is associated with reduced plasma F2-isoprostanes and urinary 20-HETE excretion in men. Free Radic Biol Med. 2007 Jun 1;42(11):1730-5. Epub 2007 Mar 12. [PubMed ]
HMDB04400	5-Acetylamino-6-amino-3-methyluracil	Urine	Nyeki A, Biollaz J, Kesselring UW, Decosterd LA: Extractionless method for the simultaneous high-performance liquid chromatographic determination of urinary caffeine metabolites for N-acetyltransferase 2, cytochrome P450 1A2 and xanthine oxidase activity assessment. J Chromatogr B Biomed Sci Appl. 2001 May 5;755(1-2):73-84. [PubMed ]
HMDB06344	Alpha-N-Phenylacetyl-L-glutamine	Blood; Urine	Yang D, Beylot M, Agarwal KC, Soloviev MV, Brunengraber H: Assay of the human liver citric acid cycle probe phenylacetylglutamine and of phenylacetate in plasma by gas chromatography-mass spectrometry. Anal Biochem. 1993 Jul;212(1):277-82. [PubMed ]
HMDB00053	Androstenedione	Blood; CSF; Urine	Reichman ME, Judd JT, Longcope C, Schatzkin A, Clevidence BA, Nair PP, Campbell WS, Taylor PR: Effects of alcohol consumption on plasma and urinary hormone concentrations in premenopausal women. J Natl Cancer Inst. 1993 May 5;85(9):722-7. [PubMed ]
HMDB01870	Benzoic acid	Urine	Geigy Scientific Tables, 8th Rev edition, pp. 130. Edited by C. Lentner, West Cadwell, N.J.: Medical education Div., Ciba-Geigy Corp. Basel, Switzerland c1981-1992.
HMDB02500	Bromide	Blood; CSF; Urine	Geigy Scientific Tables, 8th Rev edition, pp. 165-177. Edited by C. Lentner, West Cadwell, N.J.: Medical education Div., Ciba-Geigy Corp., Basel, Switzerland c1981-1992.
HMDB02322	Cadaverine	Blood; Urine	Guo K, Li L: Differential (12)C-/(13)C-Isotope Dansylation Labeling and Fast Liquid Chromatography/Mass Spectrometry for Absolute and Relative Quantification of the Metabolome. Anal Chem. 2009 Mar 23. [PubMed ]
HMDB00619	Cholic acid	Bile; Blood; Urine	Batta AK, Arora R, Salen G, Tint GS, Eskreis D, Katz S: Characterization of serum and urinary bile acids in patients with primary biliary cirrhosis by gas-liquid chromatography-mass spectrometry: effect of ursodeoxycholic acid treatment. J Lipid Res. 1989 Dec;30(12):1953-62. [PubMed ]
HMDB00570	Coproporphyrin III	Blood; Urine	Li J, Cai Z: Stacking and separation of urinary porphyrins in capillary electrophoresis: optimization of concentration efficiency and resolution. Talanta. 2008 Oct 19;77(1):331-9. Epub 2008 Jul 1. [PubMed ]
HMDB00143	D-Galactose	Blood; Cellular Cytoplasm; CSF; Urine	Geigy Scientific Tables, 8th Rev edition, pp. 130. Edited by C. Lentner, West Cadwell, N.J.: Medical education Div., Ciba-Geigy Corp. Basel, Switzerland c1981-1992.
HMDB00122	D-Glucose	Blood; Breast Milk; CSF; Saliva; Urine	Metagene: 3-METHYL-CROTONYL-GLYCINURIA
HMDB00626	Deoxycholic acid	Bile; Blood; Urine	Batta AK, Arora R, Salen G, Tint GS, Eskreis D, Katz S: Characterization of serum and urinary bile acids in patients with primary biliary cirrhosis by gas-liquid chromatography-mass spectrometry: effect of ursodeoxycholic acid treatment. J Lipid Res. 1989 Dec;30(12):1953-62. [PubMed ]
HMDB00014	Deoxycytidine	Blood; Urine	Lee SH, Jung BH, Kim SY, Chung BC: A rapid and sensitive method for quantitation of nucleosides in human urine using liquid chromatography/mass spectrometry with direct urine injection. Rapid Commun Mass Spectrom. 2004;18(9):973-7. [PubMed ]
HMDB01032	DHEA sulfate	Blood; Urine	Reichman ME, Judd JT, Longcope C, Schatzkin A, Clevidence BA, Nair PP, Campbell WS, Taylor PR: Effects of alcohol consumption on plasma and urinary hormone concentrations in premenopausal women. J Natl Cancer Inst. 1993 May 5;85(9):722-7. [PubMed ]
HMDB00575	DL-Homocystine	Blood; Urine	Metagene: HOMOCYSTINURIA DUE TO DEFECT OF N(5,10)-METHYLENE THF DEFICIENCY
HMDB00151	Estradiol	Blood; CSF; Saliva; Urine	Reichman ME, Judd JT, Longcope C, Schatzkin A, Clevidence BA, Nair PP, Campbell WS, Taylor PR: Effects of alcohol consumption on plasma and urinary hormone concentrations in premenopausal women. J Natl Cancer Inst. 1993 May 5;85(9):722-7. [PubMed ]
HMDB00153	Estriol	Amniotic Fluid; Blood; Urine	Reichman ME, Judd JT, Longcope C, Schatzkin A, Clevidence BA, Nair PP, Campbell WS, Taylor PR: Effects of alcohol consumption on plasma and urinary hormone concentrations in premenopausal women. J Natl Cancer Inst. 1993 May 5;85(9):722-7. [PubMed ]
HMDB00145	Estrone	Blood; Urine	Reichman ME, Judd JT, Longcope C, Schatzkin A, Clevidence BA, Nair PP, Campbell WS, Taylor PR: Effects of alcohol consumption on plasma and urinary hormone concentrations in premenopausal women. J Natl Cancer Inst. 1993 May 5;85(9):722-7. [PubMed ]
HMDB01425	Estrone sulfate	Blood; Urine	Reichman ME, Judd JT, Longcope C, Schatzkin A, Clevidence BA, Nair PP, Campbell WS, Taylor PR: Effects of alcohol consumption on plasma and urinary hormone concentrations in premenopausal women. J Natl Cancer Inst. 1993 May 5;85(9):722-7. [PubMed ]
HMDB00662	Fluorine	Blood; Urine	Geigy Scientific Tables, 8th Rev edition, pp. 165-177. Edited by C. Lentner, West Cadwell, N.J.: Medical education Div., Ciba-Geigy Corp., Basel, Switzerland c1981-1992.
HMDB00142	Formic acid	Blood; CSF; Urine	Moore DF, Bentley AM, Dawling S, Hoare AM, Henry JA: Folinic acid and enhanced renal elimination in formic acid intoxication. J Toxicol Clin Toxicol. 1994;32(2):199-204. [PubMed ]
HMDB00138	Glycocholic acid	Blood; Urine	Nittono H, Obinata K, Nakatsu N, Watanabe T, Niijima S, Sasaki H, Arisaka O, Kato H, Yabuta K, Miyano T: Sulfated and nonsulfated bile acids in urine of patients with biliary atresia: analysis of bile acids by high-performance liquid chromatography. J Pediatr Gastroenterol Nutr. 1986 Jan;5(1):23-9. [PubMed ]
HMDB00132	Guanine	Cellular Cytoplasm; Urine	Ohdoi C, Nyhan WL, Kuhara T: Chemical diagnosis of Lesch-Nyhan syndrome using gas chromatography-mass spectrometry detection. J Chromatogr B Analyt Technol Biomed Life Sci. 2003 Jul 15;792(1):123-30. [PubMed ]
HMDB05994	Hexanal	CSF	Goldberg EM, Blendis LM, Sandler S: A gas chromatographic--mass spectrometric study of profiles of volatile metabolites in hepatic encephalopathy. J Chromatogr. 1981 Dec 11;226(2):291-9. [PubMed ]
HMDB00755	Hydroxyphenyllactic acid	Blood; CSF; Urine	Monch E, Kneer J, Jakobs C, Arnold M, Diehl H, Batzler U: Examination of urine metabolites in the newborn period and during protein loading tests at 6 months of age--Part 1. Eur J Pediatr. 1990;149 Suppl 1:S17-24. [PubMed ]
HMDB12238	Iodide	Urine	Geigy Scientific Tables, 8th Rev edition, pp. 130. Edited by C. Lentner, West Cadwell, N.J.: Medical education Div., Ciba-Geigy Corp. Basel, Switzerland c1981-1992.
HMDB00191	L-Aspartic acid	Blood; CSF; Saliva; Urine	Guo K, Li L: Differential (12)C-/(13)C-Isotope Dansylation Labeling and Fast Liquid Chromatography/Mass Spectrometry for Absolute and Relative Quantification of the Metabolome. Anal Chem. 2009 Mar 23. [PubMed ]
HMDB00192	L-Cystine	Blood; CSF; Urine	Geigy Scientific Tables, 8th Rev edition, pp. 165-177. Edited by Cornelius Lentner. West Cadwell, N.J. : Medical education Div., Ciba-Geigy Corp. Basel, Switzerland c1981-1992.
HMDB00641	L-Glutamine	Blood; CSF; Saliva; Urine	Guo K, Li L: Differential (12)C-/(13)C-Isotope Dansylation Labeling and Fast Liquid Chromatography/Mass Spectrometry for Absolute and Relative Quantification of the Metabolome. Anal Chem. 2009 Mar 23. [PubMed ]
HMDB00190	L-Lactic acid	Bile; Blood; Cellular Cytoplasm; CSF; Urine	Geigy Scientific Tables, 8th Rev edition, pp. 130. Edited by C. Lentner, West Cadwell, N.J.: Medical education Div., Ciba-Geigy Corp. Basel, Switzerland c1981-1992.
HMDB00156	L-Malic acid	Blood; Cellular Cytoplasm; CSF; Urine	Guneral F, Bachmann C: Age-related reference values for urinary organic acids in a healthy Turkish pediatric population. Clin Chem. 1994 Jun;40(6):862-6. [PubMed ]
HMDB00696	L-Methionine	Blood; CSF; Urine	Do KQ, Lauer CJ, Schreiber W, Zollinger M, Gutteck-Amsler U, Cuenod M, Holsboer F: gamma-Glutamylglutamine and taurine concentrations are decreased in the cerebrospinal fluid of drug-naive patients with schizophrenic disorders. J Neurochem. 1995 Dec;65(6):2652-62. [PubMed ]
HMDB06112	Malondialdehyde	Blood; CSF; Urine	Agarwal R, Chase SD: Rapid, fluorimetric-liquid chromatographic determination of malondialdehyde in biological samples. J Chromatogr B Analyt Technol Biomed Life Sci. 2002 Jul 25;775(1):121-6. [PubMed ]
HMDB00752	Methylglutaric acid	Urine	Geigy Scientific Tables, 8th Rev edition, pp. 130. Edited by C. Lentner, West Cadwell, N.J.: Medical education Div., Ciba-Geigy Corp. Basel, Switzerland c1981-1992.
HMDB03502	Myo-inositol hexakisphosphate	Blood; Urine	March JG, Simonet BM, Grases F: Determination of phytic acid by gas chromatography-mass spectroscopy: application to biological samples. J Chromatogr B Biomed Sci Appl. 2001 Jun 15;757(2):247-55. [PubMed ]
HMDB01067	N-Acetylaspartylglutamic acid	Blood; CSF; Urine	Miyake M, Morino H, Mizobuchi M, Kakimoto Y: N-acetyl-L-aspartic acid, N-acetyl-alpha-L-aspartyl-L-glutamic acid and beta-citryl-L-glutamic acid in human urine. Clin Chim Acta. 1982 Mar 26;120(1):119-26. [PubMed ]
HMDB00788	Orotidine	Blood; Urine	Geigy Scientific Tables, 8th Rev edition, pp. 130. Edited by C. Lentner, West Cadwell, N.J.: Medical education Div., Ciba-Geigy Corp. Basel, Switzerland c1981-1992.
HMDB00208	Oxoglutaric acid	Blood; Cellular Cytoplasm; CSF; N/A; Urine	Metagene: 2-KETOGLUTARATE DEHYDROGENASE COMPLEX DEFICIENCY
HMDB01830	Progesterone	Blood; CSF	Reichman ME, Judd JT, Longcope C, Schatzkin A, Clevidence BA, Nair PP, Campbell WS, Taylor PR: Effects of alcohol consumption on plasma and urinary hormone concentrations in premenopausal women. J Natl Cancer Inst. 1993 May 5;85(9):722-7. [PubMed ]
HMDB00840	Salicyluric acid	Blood; Urine
HMDB03966	Selenomethionine	Blood; Urine	Elmore E, Siddiqui S, Navidi M, Steele VE, Redpath JL: Correlation of in vitro chemopreventive efficacy data from the human epidermal cell assay with animal efficacy data and clinical trial plasma levels. J Cell Biochem. 2005 Jun 1;95(3):571-88. [PubMed ]
HMDB00262	Thymine	Blood; CSF; Urine	Metagene: DIHYDROPYRIMIDINE DEHYDROGENASE DEFICIENCY (DHPD)
HMDB00289	Uric acid	Amniotic Fluid; Bile; Blood; CSF; Saliva; Urine	Geigy Scientific Tables, 8th Rev edition, pp. 130. Edited by C. Lentner, West Cadwell, N.J.: Medical education Div., Ciba-Geigy Corp. Basel, Switzerland c1981-1992.
HMDB11714	Vanilpyruvic acid	Blood; Urine	Sreekumar A, Poisson LM, Rajendiran TM, Khan AP, Cao Q, Yu J, Laxman B, Mehra R, Lonigro RJ, Li Y, Nyati MK, Ahsan A, Kalyana-Sundaram S, Han B, Cao X, Byun J, Omenn GS, Ghosh D, Pennathur S, Alexander DC, Berger A, Shuster JR, Wei JT, Varambally S, Beecher C, Chinnaiyan AM: Metabolomic profiles delineate potential role for sarcosine in prostate cancer progression. Nature. 2009 Feb 12;457(7231):910-4. [PubMed ]

ACTH Deficiency (Isolated) (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00063	Cortisol	Blood; CSF; Saliva; Urine	Metagene: ACTH DEFICIENCY, ISOLATED

Active tobacco user (5 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB04350	Anabasine	Blood; Urine	Moyer TP, Charlson JR, Enger RJ, Dale LC, Ebbert JO, Schroeder DR, Hurt RD: Simultaneous analysis of nicotine, nicotine metabolites, and tobacco alkaloids in serum or urine by tandem mass spectrometry, with clinically relevant metabolic profiles. Clin Chem. 2002 Sep;48(9):1460-71. [PubMed ]
HMDB01046	Cotinine	Blood; Saliva; Urine	Moyer TP, Charlson JR, Enger RJ, Dale LC, Ebbert JO, Schroeder DR, Hurt RD: Simultaneous analysis of nicotine, nicotine metabolites, and tobacco alkaloids in serum or urine by tandem mass spectrometry, with clinically relevant metabolic profiles. Clin Chem. 2002 Sep;48(9):1460-71. [PubMed ]
HMDB01390	Hydroxycotinine	Blood; Saliva; Urine	Moyer TP, Charlson JR, Enger RJ, Dale LC, Ebbert JO, Schroeder DR, Hurt RD: Simultaneous analysis of nicotine, nicotine metabolites, and tobacco alkaloids in serum or urine by tandem mass spectrometry, with clinically relevant metabolic profiles. Clin Chem. 2002 Sep;48(9):1460-71. [PubMed ]
HMDB01934	L(-)-Nicotine pestanal	Blood; Urine	Moyer TP, Charlson JR, Enger RJ, Dale LC, Ebbert JO, Schroeder DR, Hurt RD: Simultaneous analysis of nicotine, nicotine metabolites, and tobacco alkaloids in serum or urine by tandem mass spectrometry, with clinically relevant metabolic profiles. Clin Chem. 2002 Sep;48(9):1460-71. [PubMed ]
HMDB01126	Nornicotine	Urine	Moyer TP, Charlson JR, Enger RJ, Dale LC, Ebbert JO, Schroeder DR, Hurt RD: Simultaneous analysis of nicotine, nicotine metabolites, and tobacco alkaloids in serum or urine by tandem mass spectrometry, with clinically relevant metabolic profiles. Clin Chem. 2002 Sep;48(9):1460-71. [PubMed ]

Acute ethanol intoxication (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00190	L-Lactic acid	Bile; Blood; Cellular Cytoplasm; CSF; Urine	Fulop M, Bock J, Ben-Ezra J, Antony M, Danzig J, Gage JS: Plasma lactate and 3-hydroxybutyrate levels in patients with acute ethanol intoxication. Am J Med. 1986 Feb;80(2):191-4. [PubMed ]

Acute intermittent porphyria (3 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00643	Coproporphyrin I	Blood; Urine	Zuijderhoudt FM, Koehorst SG, Kluitenberg WE, Dorresteijn-de Bok J: On accuracy and precision of a HPLC method for measurement of urine porphyrin concentrations. Clin Chem Lab Med. 2000 Mar;38(3):227-30. [PubMed ]
HMDB00570	Coproporphyrin III	Blood; Urine	Zuijderhoudt FM, Koehorst SG, Kluitenberg WE, Dorresteijn-de Bok J: On accuracy and precision of a HPLC method for measurement of urine porphyrin concentrations. Clin Chem Lab Med. 2000 Mar;38(3):227-30. [PubMed ]
HMDB00737	Heptacarboxylporphyrin I	Urine	Zuijderhoudt FM, Koehorst SG, Kluitenberg WE, Dorresteijn-de Bok J: On accuracy and precision of a HPLC method for measurement of urine porphyrin concentrations. Clin Chem Lab Med. 2000 Mar;38(3):227-30. [PubMed ]

Acute liver disease (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB01045	Enkephalin L	Blood	Thornton JR, Losowsky MS: Plasma leucine enkephalin is increased in liver disease. Gut. 1989 Oct;30(10):1392-5. [PubMed ]

Acute myelogenous leukemia (AML) (2 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00067	Cholesterol	Bile; Blood; CSF; Saliva; Urine	Tatidis L, Vitols S, Gruber A, Paul C, Axelson M: Cholesterol catabolism in patients with acute myelogenous leukemia and hypocholesterolemia: suppressed levels of a circulating marker for bile acid synthesis. Cancer Lett. 2001 Sep 20;170(2):169-75. [PubMed ]
HMDB00122	D-Glucose	Blood; Breast Milk; CSF; Saliva; Urine	Tatidis L, Vitols S, Gruber A, Paul C, Axelson M: Cholesterol catabolism in patients with acute myelogenous leukemia and hypocholesterolemia: suppressed levels of a circulating marker for bile acid synthesis. Cancer Lett. 2001 Sep 20;170(2):169-75. [PubMed ]

Acute or chronic demyelinating polyneuropathies (2 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB01419	24-Hydroxycholesterol	Blood; CSF	Leoni V, Masterman T, Mousavi FS, Wretlind B, Wahlund LO, Diczfalusy U, Hillert J, Bjorkhem I: Diagnostic use of cerebral and extracerebral oxysterols. Clin Chem Lab Med. 2004 Feb;42(2):186-91. [PubMed ]
HMDB02103	27-Hydroxycholesterol	Blood; CSF	Leoni V, Masterman T, Mousavi FS, Wretlind B, Wahlund LO, Diczfalusy U, Hillert J, Bjorkhem I: Diagnostic use of cerebral and extracerebral oxysterols. Clin Chem Lab Med. 2004 Feb;42(2):186-91. [PubMed ]

Acute seizures (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00192	L-Cystine	Blood; CSF; Urine	Rainesalo S, Keranen T, Palmio J, Peltola J, Oja SS, Saransaari P: Plasma and cerebrospinal fluid amino acids in epileptic patients. Neurochem Res. 2004 Jan;29(1):319-24. [PubMed ]

Addison's disease (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00068	Epinephrine	Blood; CSF; Urine	Bornstein SR, Breidert M, Ehrhart-Bornstein M, Kloos B, Scherbaum WA: Plasma catecholamines in patients with Addison's disease. Clin Endocrinol (Oxf). 1995 Feb;42(2):215-8. [PubMed ]

Adenosine deaminase deficiency (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00101	Deoxyadenosine	Urine	Yamamoto T, Moriwaki Y, Takahashi S, Fujita T, Tsutsumi Z, Yamakita J, Shimizu K, Shiota M, Ohta S, Higashino K: Determination of adenosine and deoxyadenosine in urine by high-performance liquid chromatography with column switching. J Chromatogr B Biomed Sci Appl. 1998 Nov 20;719(1-2):55-61. [PubMed ]

Adenosine deaminase(ADA) deficiency (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB11599	1-Methyladenine	Urine	Mills GC: 1-Methyladenine in urine of an adenosine deaminase-deficient adult without immunodeficiency. Biochem Med Metab Biol. 1989 Dec;42(3):240-7. [PubMed ]

Adenylosuccinate lyase (ASDL) deficiency (3 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00797	SAICAR	CSF	Marinaki AM, Champion M, Kurian MA, Simmonds HA, Marie S, Vincent MF, van den Berghe G, Duley JA, Fairbanks LD: Adenylosuccinate lyase deficiency--first British case. Nucleosides Nucleotides Nucleic Acids. 2004 Oct;23(8-9):1231-3. [PubMed ]
HMDB00912	Succinyladenosine	Blood; CSF	Marinaki AM, Champion M, Kurian MA, Simmonds HA, Marie S, Vincent MF, van den Berghe G, Duley JA, Fairbanks LD: Adenylosuccinate lyase deficiency--first British case. Nucleosides Nucleotides Nucleic Acids. 2004 Oct;23(8-9):1231-3. [PubMed ]
HMDB00289	Uric acid	Amniotic Fluid; Bile; Blood; CSF; Saliva; Urine	Marinaki AM, Champion M, Kurian MA, Simmonds HA, Marie S, Vincent MF, van den Berghe G, Duley JA, Fairbanks LD: Adenylosuccinate lyase deficiency--first British case. Nucleosides Nucleotides Nucleic Acids. 2004 Oct;23(8-9):1231-3. [PubMed ]

Adrenal Hypolasia (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00063	Cortisol	Blood; CSF; Saliva; Urine	Metagene: ADRENAL HYPOPLASIA. ADDISON DISEASE, X-LINKED

Adrenoleukodystrophy (3 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB02356	Hexacosanoic acid	Blood	Metagene: ADRENOLEUKODYSTROPHY, NEONATAL
HMDB00801	Phytanic acid	Blood	Metagene: ADRENOLEUKODYSTROPHY, NEONATAL
HMDB00795	Pristanic acid	Blood	Metagene: ADRENOLEUKODYSTROPHY, NEONATAL

Adrenoleukodystrophy (ALD) (2 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00716	L-Pipecolic acid	Blood	Watkins PA, Chen WW, Harris CJ, Hoefler G, Hoefler S, Blake DC Jr, Balfe A, Kelley RI, Moser AB, Beard ME, et al.: Peroxisomal bifunctional enzyme deficiency. J Clin Invest. 1989 Mar;83(3):771-7. [PubMed ]
HMDB02163	Trihydroxycoprostanoic acid	Blood	Watkins PA, Chen WW, Harris CJ, Hoefler G, Hoefler S, Blake DC Jr, Balfe A, Kelley RI, Moser AB, Beard ME, et al.: Peroxisomal bifunctional enzyme deficiency. J Clin Invest. 1989 Mar;83(3):771-7. [PubMed ]

Adrenoleukodystrophy (X-Linked) (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00070	Pipecolic acid	Amniotic Fluid; Blood; CSF; Urine	Metagene: ADRENOLEUKODYSTROPHY, X-LINKED

Adrenomyeloneuropathy (2 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB02068	Erucic acid	Blood	Aubourg P, Adamsbaum C, Lavallard-Rousseau MC, Rocchiccioli F, Cartier N, Jambaque I, Jakobezak C, Lemaitre A, Boureau F, Wolf C, et al.: A two-year trial of oleic and erucic acids ("Lorenzo's oil") as treatment for adrenomyeloneuropathy. N Engl J Med. 1993 Sep 9;329(11):745-52. [PubMed ]
HMDB02356	Hexacosanoic acid	Blood	Sadeghi-Nejad A, Senior B: Adrenomyeloneuropathy presenting as Addison's disease in childhood. N Engl J Med. 1990 Jan 4;322(1):13-6. [PubMed ]

After 15 day of phylloquinone depletion (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB04808	7C-aglycone	Urine	Harrington DJ, Booth SL, Card DJ, Shearer MJ: Excretion of the urinary 5C- and 7C-aglycone metabolites of vitamin K by young adults responds to changes in dietary phylloquinone and dihydrophylloquinone intakes. J Nutr. 2007 Jul;137(7):1763-8. [PubMed ]

After 15 day of phylloquinone restriction (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB04810	5C-aglycone	Urine	Harrington DJ, Booth SL, Card DJ, Shearer MJ: Excretion of the urinary 5C- and 7C-aglycone metabolites of vitamin K by young adults responds to changes in dietary phylloquinone and dihydrophylloquinone intakes. J Nutr. 2007 Jul;137(7):1763-8. [PubMed ]

After alcohol intake (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB05199	(R)-Salsolinol	Blood; CSF	Haber H, Winkler A, Putscher I, Henklein P, Baeger I, Georgi M, Melzig MF: Plasma and urine salsolinol in humans: effect of acute ethanol intake on the enantiomeric composition of salsolinol. Alcohol Clin Exp Res. 1996 Feb;20(1):87-92. [PubMed ]

After tomato drink intake (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB03993	Alpha-Carotene	Blood	Porrini M, Riso P, Brusamolino A, Berti C, Guarnieri S, Visioli F: Daily intake of a formulated tomato drink affects carotenoid plasma and lymphocyte concentrations and improves cellular antioxidant protection. Br J Nutr. 2005 Jan;93(1):93-9. [PubMed ]

AIDS (6 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB01396	5-Methyltetrahydrofolic acid	Blood; CSF	Surtees R, Hyland K, Smith I: Central-nervous-system methyl-group metabolism in children with neurological complications of HIV infection. Lancet. 1990 Mar 17;335(8690):619-21. [PubMed ]
HMDB00039	Butyric acid	Blood; CSF; Saliva; Urine	Stein TP, Koerner B, Schluter MD, Leskiw MJ, Gaprindachvilli T, Richards EW, Cope FO, Condolucci D: Weight loss, the gut and the inflammatory response in aids patients. Cytokine. 1997 Feb;9(2):143-7. [PubMed ]
HMDB00574	L-Cysteine	Blood; CSF; Urine	Naisbitt DJ, Vilar FJ, Stalford AC, Wilkins EG, Pirmohamed M, Park BK: Plasma cysteine deficiency and decreased reduction of nitrososulfamethoxazole with HIV infection. AIDS Res Hum Retroviruses. 2000 Dec 10;16(18):1929-38. [PubMed ]
HMDB00765	Mannitol	Blood; CSF; Urine	Megson GM, Stevens DA, Hamilton JR, Denning DW: D-mannitol in cerebrospinal fluid of patients with AIDS and cryptococcal meningitis. J Clin Microbiol. 1996 Jan;34(1):218-21. [PubMed ]
HMDB00845	Neopterin	Blood; CSF; Urine	Ellaurie M, Calvelli T, Rubinstein A: Neopterin concentrations in pediatric human immunodeficiency virus infection as predictor of disease activity. Pediatr Infect Dis J. 1992 Apr;11(4):286-9. [PubMed ]
HMDB00232	Quinolinic acid	Blood; CSF; Urine	Heyes MP, Saito K, Lackner A, Wiley CA, Achim CL, Markey SP: Sources of the neurotoxin quinolinic acid in the brain of HIV-1-infected patients and retrovirus-infected macaques. FASEB J. 1998 Jul;12(10):881-96. [PubMed ]

Alcohol intoxication (6 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB11469	2-Butanol	Blood	Jones AW, Lund M, Andersson E: Drinking drivers in Sweden who consume denatured alcohol preparations: an analytical-toxicological study. J Anal Toxicol. 1989 Jul-Aug;13(4):199-203. [PubMed ]
HMDB01659	Acetone	Blood; CSF; Urine	Jones AW, Lund M, Andersson E: Drinking drivers in Sweden who consume denatured alcohol preparations: an analytical-toxicological study. J Anal Toxicol. 1989 Jul-Aug;13(4):199-203. [PubMed ]
HMDB00474	Butanone	Blood; Urine	Jones AW, Lund M, Andersson E: Drinking drivers in Sweden who consume denatured alcohol preparations: an analytical-toxicological study. J Anal Toxicol. 1989 Jul-Aug;13(4):199-203. [PubMed ]
HMDB00108	Ethanol	Blood; CSF; Saliva; Urine	Jones AW, Lund M, Andersson E: Drinking drivers in Sweden who consume denatured alcohol preparations: an analytical-toxicological study. J Anal Toxicol. 1989 Jul-Aug;13(4):199-203. [PubMed ]
HMDB00863	Isopropyl alcohol	Blood; CSF	Jones AW, Lund M, Andersson E: Drinking drivers in Sweden who consume denatured alcohol preparations: an analytical-toxicological study. J Anal Toxicol. 1989 Jul-Aug;13(4):199-203. [PubMed ]
HMDB01875	Methanol	Blood; CSF; Saliva; Urine	Jones AW, Lund M, Andersson E: Drinking drivers in Sweden who consume denatured alcohol preparations: an analytical-toxicological study. J Anal Toxicol. 1989 Jul-Aug;13(4):199-203. [PubMed ]

Alcoholism (7 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00990	Acetaldehyde	Blood; CSF; Urine	Brecher AS, Adamu MT: Short- and long-term effects of acetaldehyde on plasma. Alcohol. 2002 Jan;26(1):49-53. [PubMed ]
HMDB01875	Methanol	Blood; CSF; Saliva; Urine	Jones AW, Lowinger H: Relationship between the concentration of ethanol and methanol in blood samples from Swedish drinking drivers. Forensic Sci Int. 1988 Jun;37(4):277-85. [PubMed ]
HMDB01488	Nicotinic acid	Blood; CSF; Urine	Dastur DK, Santhadevi N, Quadros EV, Avari FC, Wadia NH, Desai MN, Bharucha EP: The B-vitamins in malnutrition with alcoholism. A model of intervitamin relationships. Br J Nutr. 1976 Sep;36(2):143-59. [PubMed ]
HMDB00210	Pantothenic acid	Blood; CSF; Urine	Dastur DK, Santhadevi N, Quadros EV, Avari FC, Wadia NH, Desai MN, Bharucha EP: The B-vitamins in malnutrition with alcoholism. A model of intervitamin relationships. Br J Nutr. 1976 Sep;36(2):143-59. [PubMed ]
HMDB00244	Riboflavin	Blood; CSF; Urine	Dastur DK, Santhadevi N, Quadros EV, Avari FC, Wadia NH, Desai MN, Bharucha EP: The B-vitamins in malnutrition with alcoholism. A model of intervitamin relationships. Br J Nutr. 1976 Sep;36(2):143-59. [PubMed ]
HMDB06088	Scyllitol	Blood; CSF	Viola A, Nicoli F, Denis B, Confort-Gouny S, Le Fur Y, Ranjeva JP, Viout P, Cozzone PJ: High cerebral scyllo-inositol: a new marker of brain metabolism disturbances induced by chronic alcoholism. MAGMA. 2004 Sep;17(1):47-61. Epub 2004 Aug 31. [PubMed ]
HMDB00235	Thiamine	Blood; CSF; Urine	Dastur DK, Santhadevi N, Quadros EV, Avari FC, Wadia NH, Desai MN, Bharucha EP: The B-vitamins in malnutrition with alcoholism. A model of intervitamin relationships. Br J Nutr. 1976 Sep;36(2):143-59. [PubMed ]

Aldehyde dehydrogenase deficiency (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00990	Acetaldehyde	Blood; CSF; Urine	Brecher AS, Adamu MT: Short- and long-term effects of acetaldehyde on plasma. Alcohol. 2002 Jan;26(1):49-53. [PubMed ]

Alkaptonuria (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00130	Homogentisic acid	Blood; Urine	Phornphutkul C, Introne WJ, Perry MB, Bernardini I, Murphey MD, Fitzpatrick DL, Anderson PD, Huizing M, Anikster Y, Gerber LH, Gahl WA: Natural history of alkaptonuria. N Engl J Med. 2002 Dec 26;347(26):2111-21. [PubMed ]

Alpha-1-antitrypsin deficiency (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00138	Glycocholic acid	Blood; Urine	Matsui A, Psacharopoulos HT, Mowat AP, Portmann B, Murphy GM: Radioimmunoassay of serum glycocholic acid, standard laboratory tests of liver function and liver biopsy findings: comparative study of children with liver disease. J Clin Pathol. 1982 Sep;35(9):1011-7. [PubMed ]

Alpha-aminoadipic aciduria (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00510	Aminoadipic acid	Blood; Urine	Vallat C, Rivier F, Bellet H, Magnan de Bornier B, Mion H, Echenne B: Treatment with vigabatrin may mimic alpha-aminoadipic aciduria. Epilepsia. 1996 Aug;37(8):803-5. [PubMed ]

Alzheimer's diesease (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00177	L-Histidine	Blood; CSF; Saliva; Urine	Fonteh AN, Harrington RJ, Tsai A, Liao P, Harrington MG: Free amino acid and dipeptide changes in the body fluids from Alzheimer's disease subjects. Amino Acids. 2007 Feb;32(2):213-24. Epub 2006 Oct 10. [PubMed ]

Alzheimer's disease (85 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB02712	1,5-Anhydrosorbitol	Blood; CSF	Shetty HU, Holloway HW, Schapiro MB: Cerebrospinal fluid and plasma distribution of myo-inositol and other polyols in Alzheimer disease. Clin Chem. 1996 Feb;42(2):298-302. [PubMed ]
HMDB00001	1-Methylhistidine	Blood; CSF; Saliva; Urine	Fonteh AN, Harrington RJ, Tsai A, Liao P, Harrington MG: Free amino acid and dipeptide changes in the body fluids from Alzheimer's disease subjects. Amino Acids. 2007 Feb;32(2):213-24. Epub 2006 Oct 10. [PubMed ]
HMDB01419	24-Hydroxycholesterol	Blood; CSF	Leoni V, Masterman T, Mousavi FS, Wretlind B, Wahlund LO, Diczfalusy U, Hillert J, Bjorkhem I: Diagnostic use of cerebral and extracerebral oxysterols. Clin Chem Lab Med. 2004 Feb;42(2):186-91. [PubMed ]
HMDB02103	27-Hydroxycholesterol	Blood; CSF	Leoni V, Masterman T, Mousavi FS, Wretlind B, Wahlund LO, Diczfalusy U, Hillert J, Bjorkhem I: Diagnostic use of cerebral and extracerebral oxysterols. Clin Chem Lab Med. 2004 Feb;42(2):186-91. [PubMed ]
HMDB01336	3,4-Dihydroxybenzeneacetic acid	Blood; CSF; Urine	Raskind MA, Peskind ER, Holmes C, Goldstein DS: Patterns of cerebrospinal fluid catechols support increased central noradrenergic responsiveness in aging and Alzheimer's disease. Biol Psychiatry. 1999 Sep 15;46(6):756-65. [PubMed ]
HMDB00479	3-Methylhistidine	Blood; CSF; Urine	Fonteh AN, Harrington RJ, Tsai A, Liao P, Harrington MG: Free amino acid and dipeptide changes in the body fluids from Alzheimer's disease subjects. Amino Acids. 2007 Feb;32(2):213-24. Epub 2006 Oct 10. [PubMed ]
HMDB06055	4-Hydroxy-L-proline	Blood; CSF; Urine	Fonteh AN, Harrington RJ, Tsai A, Liao P, Harrington MG: Free amino acid and dipeptide changes in the body fluids from Alzheimer's disease subjects. Amino Acids. 2007 Feb;32(2):213-24. Epub 2006 Oct 10. [PubMed ]
HMDB04362	4-Hydroxynonenal	Blood; CSF; Urine	Selley ML, Close DR, Stern SE: The effect of increased concentrations of homocysteine on the concentration of (E)-4-hydroxy-2-nonenal in the plasma and cerebrospinal fluid of patients with Alzheimer's disease. Neurobiol Aging. 2002 May-Jun;23(3):383-8. [PubMed ]
HMDB00450	5-Hydroxylysine	Blood; Urine	Fonteh AN, Harrington RJ, Tsai A, Liao P, Harrington MG: Free amino acid and dipeptide changes in the body fluids from Alzheimer's disease subjects. Amino Acids. 2007 Feb;32(2):213-24. Epub 2006 Oct 10. [PubMed ]
HMDB02032	8-Hydroxyguanine	Blood; CSF	Lovell MA, Markesbery WR: Ratio of 8-hydroxyguanine in intact DNA to free 8-hydroxyguanine is increased in Alzheimer disease ventricular cerebrospinal fluid. Arch Neurol. 2001 Mar;58(3):392-6. [PubMed ]
HMDB02044	8-Hydroxyguanosine	Blood; CSF	Abe T, Tohgi H, Isobe C, Murata T, Sato C: Remarkable increase in the concentration of 8-hydroxyguanosine in cerebrospinal fluid from patients with Alzheimer's disease. J Neurosci Res. 2002 Nov 1;70(3):447-50. [PubMed ]
HMDB00895	Acetylcholine	CSF; Urine	Jia JP, Jia JM, Zhou WD, Xu M, Chu CB, Yan X, Sun YX: Differential acetylcholine and choline concentrations in the cerebrospinal fluid of patients with Alzheimer's disease and vascular dementia. Chin Med J (Engl). 2004 Aug;117(8):1161-4. [PubMed ]
HMDB01247	Aluminum	Blood; CSF	Bocca B, Forte G, Petrucci F, Pino A, Marchione F, Bomboi G, Senofonte O, Giubilei F, Alimonti A: Monitoring of chemical elements and oxidative damage in patients affected by Alzheimer's disease. Ann Ist Super Sanita. 2005;41(2):197-203. [PubMed ]
HMDB00194	Anserine	Blood; Urine	Fonteh AN, Harrington RJ, Tsai A, Liao P, Harrington MG: Free amino acid and dipeptide changes in the body fluids from Alzheimer's disease subjects. Amino Acids. 2007 Feb;32(2):213-24. Epub 2006 Oct 10. [PubMed ]
HMDB04142	Barium	Blood; CSF; Urine	Bocca B, Forte G, Petrucci F, Pino A, Marchione F, Bomboi G, Senofonte O, Giubilei F, Alimonti A: Monitoring of chemical elements and oxidative damage in patients affected by Alzheimer's disease. Ann Ist Super Sanita. 2005;41(2):197-203. [PubMed ]
HMDB02387	Beryllium	Blood	Bocca B, Forte G, Petrucci F, Pino A, Marchione F, Bomboi G, Senofonte O, Giubilei F, Alimonti A: Monitoring of chemical elements and oxidative damage in patients affected by Alzheimer's disease. Ann Ist Super Sanita. 2005;41(2):197-203. [PubMed ]
HMDB02196	Bismuth	Blood	Bocca B, Forte G, Petrucci F, Pino A, Marchione F, Bomboi G, Senofonte O, Giubilei F, Alimonti A: Monitoring of chemical elements and oxidative damage in patients affected by Alzheimer's disease. Ann Ist Super Sanita. 2005;41(2):197-203. [PubMed ]
HMDB03638	Cadmium	Blood; CSF; Urine	Bocca B, Forte G, Petrucci F, Pino A, Marchione F, Bomboi G, Senofonte O, Giubilei F, Alimonti A: Monitoring of chemical elements and oxidative damage in patients affected by Alzheimer's disease. Ann Ist Super Sanita. 2005;41(2):197-203. [PubMed ]
HMDB00464	Calcium	Blood; CSF; Urine	Bocca B, Forte G, Petrucci F, Pino A, Marchione F, Bomboi G, Senofonte O, Giubilei F, Alimonti A: Monitoring of chemical elements and oxidative damage in patients affected by Alzheimer's disease. Ann Ist Super Sanita. 2005;41(2):197-203. [PubMed ]
HMDB00033	Carnosine	Blood; Urine	Fonteh AN, Harrington RJ, Tsai A, Liao P, Harrington MG: Free amino acid and dipeptide changes in the body fluids from Alzheimer's disease subjects. Amino Acids. 2007 Feb;32(2):213-24. Epub 2006 Oct 10. [PubMed ]
HMDB00097	Choline	Blood; Breast Milk; CSF; Saliva; Urine	Walter A, Korth U, Hilgert M, Hartmann J, Weichel O, Hilgert M, Fassbender K, Schmitt A, Klein J: Glycerophosphocholine is elevated in cerebrospinal fluid of Alzheimer patients. Neurobiol Aging. 2004 Nov-Dec;25(10):1299-303. [PubMed ]
HMDB00904	Citrulline	Blood; CSF; Urine	Fonteh AN, Harrington RJ, Tsai A, Liao P, Harrington MG: Free amino acid and dipeptide changes in the body fluids from Alzheimer's disease subjects. Amino Acids. 2007 Feb;32(2):213-24. Epub 2006 Oct 10. [PubMed ]
HMDB00608	Cobalt	Blood; CSF; Urine	Bocca B, Forte G, Petrucci F, Pino A, Marchione F, Bomboi G, Senofonte O, Giubilei F, Alimonti A: Monitoring of chemical elements and oxidative damage in patients affected by Alzheimer's disease. Ann Ist Super Sanita. 2005;41(2):197-203. [PubMed ]
HMDB00657	Copper	Blood; CSF; Urine	Molina JA, Jimenez-Jimenez FJ, Aguilar MV, Meseguer I, Mateos-Vega CJ, Gonzalez-Munoz MJ, de Bustos F, Porta J, Orti-Pareja M, Zurdo M, Barrios E, Martinez-Para MC: Cerebrospinal fluid levels of transition metals in patients with Alzheimer's disease. J Neural Transm. 1998;105(4-5):479-88. [PubMed ]
HMDB00122	D-Glucose	Blood; Breast Milk; CSF; Saliva; Urine	Redjems-Bennani N, Jeandel C, Lefebvre E, Blain H, Vidailhet M, Gueant JL: Abnormal substrate levels that depend upon mitochondrial function in cerebrospinal fluid from Alzheimer patients. Gerontology. 1998;44(5):300-4. [PubMed ]
HMDB00073	Dopamine	Blood; CSF; Urine	Fonteh AN, Harrington RJ, Tsai A, Liao P, Harrington MG: Free amino acid and dipeptide changes in the body fluids from Alzheimer's disease subjects. Amino Acids. 2007 Feb;32(2):213-24. Epub 2006 Oct 10. [PubMed ]
HMDB03869	Epsilon-(gamma-Glutamyl)-lysine	Blood; CSF	Fonteh AN, Harrington RJ, Tsai A, Liao P, Harrington MG: Free amino acid and dipeptide changes in the body fluids from Alzheimer's disease subjects. Amino Acids. 2007 Feb;32(2):213-24. Epub 2006 Oct 10. [PubMed ]
HMDB00121	Folic acid	Blood; Cellular Cytoplasm; CSF; Urine	Selley ML, Close DR, Stern SE: The effect of increased concentrations of homocysteine on the concentration of (E)-4-hydroxy-2-nonenal in the plasma and cerebrospinal fluid of patients with Alzheimer's disease. Neurobiol Aging. 2002 May-Jun;23(3):383-8. [PubMed ]
HMDB00134	Fumaric acid	Blood; CSF; Urine	Redjems-Bennani N, Jeandel C, Lefebvre E, Blain H, Vidailhet M, Gueant JL: Abnormal substrate levels that depend upon mitochondrial function in cerebrospinal fluid from Alzheimer patients. Gerontology. 1998;44(5):300-4. [PubMed ]
HMDB00107	Galactitol	Amniotic Fluid; Blood; CSF; Urine	Shetty HU, Holloway HW, Schapiro MB: Cerebrospinal fluid and plasma distribution of myo-inositol and other polyols in Alzheimer disease. Clin Chem. 1996 Feb;42(2):298-302. [PubMed ]
HMDB00112	Gamma-Aminobutyric acid	Blood; CSF; Saliva; Urine	Fonteh AN, Harrington RJ, Tsai A, Liao P, Harrington MG: Free amino acid and dipeptide changes in the body fluids from Alzheimer's disease subjects. Amino Acids. 2007 Feb;32(2):213-24. Epub 2006 Oct 10. [PubMed ]
HMDB00086	Glycerophosphocholine	Blood; Breast Milk; CSF; Semen	Walter A, Korth U, Hilgert M, Hartmann J, Weichel O, Hilgert M, Fassbender K, Schmitt A, Klein J: Glycerophosphocholine is elevated in cerebrospinal fluid of Alzheimer patients. Neurobiol Aging. 2004 Nov-Dec;25(10):1299-303. [PubMed ]
HMDB00123	Glycine	Bile; Blood; CSF; Saliva; Urine	Fonteh AN, Harrington RJ, Tsai A, Liao P, Harrington MG: Free amino acid and dipeptide changes in the body fluids from Alzheimer's disease subjects. Amino Acids. 2007 Feb;32(2):213-24. Epub 2006 Oct 10. [PubMed ]
HMDB00721	Glycylproline	Blood; CSF; Urine	Fonteh AN, Harrington RJ, Tsai A, Liao P, Harrington MG: Free amino acid and dipeptide changes in the body fluids from Alzheimer's disease subjects. Amino Acids. 2007 Feb;32(2):213-24. Epub 2006 Oct 10. [PubMed ]
HMDB00742	Homocysteine	Blood; CSF; Urine	Serot JM, Barbe F, Arning E, Bottiglieri T, Franck P, Montagne P, Nicolas JP: Homocysteine and methylmalonic acid concentrations in cerebrospinal fluid: relation with age and Alzheimer's disease. J Neurol Neurosurg Psychiatry. 2005 Nov;76(11):1585-7. [PubMed ]
HMDB00725	Hydroxyproline	Blood; Urine	Fonteh AN, Harrington RJ, Tsai A, Liao P, Harrington MG: Free amino acid and dipeptide changes in the body fluids from Alzheimer's disease subjects. Amino Acids. 2007 Feb;32(2):213-24. Epub 2006 Oct 10. [PubMed ]
HMDB00692	Iron	Blood; CSF; Urine	Molina JA, Jimenez-Jimenez FJ, Aguilar MV, Meseguer I, Mateos-Vega CJ, Gonzalez-Munoz MJ, de Bustos F, Porta J, Orti-Pareja M, Zurdo M, Barrios E, Martinez-Para MC: Cerebrospinal fluid levels of transition metals in patients with Alzheimer's disease. J Neural Transm. 1998;105(4-5):479-88. [PubMed ]
HMDB06284	L-2,4-diaminobutyric acid	Blood; Urine	Fonteh AN, Harrington RJ, Tsai A, Liao P, Harrington MG: Free amino acid and dipeptide changes in the body fluids from Alzheimer's disease subjects. Amino Acids. 2007 Feb;32(2):213-24. Epub 2006 Oct 10. [PubMed ]
HMDB00452	L-Alpha-aminobutyric acid	Blood; CSF; Urine	Fonteh AN, Harrington RJ, Tsai A, Liao P, Harrington MG: Free amino acid and dipeptide changes in the body fluids from Alzheimer's disease subjects. Amino Acids. 2007 Feb;32(2):213-24. Epub 2006 Oct 10. [PubMed ]
HMDB01851	L-Arabitol	Blood; CSF; Urine	Shetty HU, Holloway HW, Schapiro MB: Cerebrospinal fluid and plasma distribution of myo-inositol and other polyols in Alzheimer disease. Clin Chem. 1996 Feb;42(2):298-302. [PubMed ]
HMDB00517	L-Arginine	Blood; CSF; Urine	Fonteh AN, Harrington RJ, Tsai A, Liao P, Harrington MG: Free amino acid and dipeptide changes in the body fluids from Alzheimer's disease subjects. Amino Acids. 2007 Feb;32(2):213-24. Epub 2006 Oct 10. [PubMed ]
HMDB00168	L-Asparagine	Blood; CSF; Urine	Fonteh AN, Harrington RJ, Tsai A, Liao P, Harrington MG: Free amino acid and dipeptide changes in the body fluids from Alzheimer's disease subjects. Amino Acids. 2007 Feb;32(2):213-24. Epub 2006 Oct 10. [PubMed ]
HMDB00191	L-Aspartic acid	Blood; CSF; Saliva; Urine	Fonteh AN, Harrington RJ, Tsai A, Liao P, Harrington MG: Free amino acid and dipeptide changes in the body fluids from Alzheimer's disease subjects. Amino Acids. 2007 Feb;32(2):213-24. Epub 2006 Oct 10. [PubMed ]
HMDB00099	L-Cystathionine	Blood; CSF; Urine	Fonteh AN, Harrington RJ, Tsai A, Liao P, Harrington MG: Free amino acid and dipeptide changes in the body fluids from Alzheimer's disease subjects. Amino Acids. 2007 Feb;32(2):213-24. Epub 2006 Oct 10. [PubMed ]
HMDB00192	L-Cystine	Blood; CSF; Urine	Fonteh AN, Harrington RJ, Tsai A, Liao P, Harrington MG: Free amino acid and dipeptide changes in the body fluids from Alzheimer's disease subjects. Amino Acids. 2007 Feb;32(2):213-24. Epub 2006 Oct 10. [PubMed ]
HMDB00181	L-Dopa	Blood; CSF; Urine	Fonteh AN, Harrington RJ, Tsai A, Liao P, Harrington MG: Free amino acid and dipeptide changes in the body fluids from Alzheimer's disease subjects. Amino Acids. 2007 Feb;32(2):213-24. Epub 2006 Oct 10. [PubMed ]
HMDB00148	L-Glutamic acid	Blood; Cellular Cytoplasm; CSF; Saliva; Urine	Fonteh AN, Harrington RJ, Tsai A, Liao P, Harrington MG: Free amino acid and dipeptide changes in the body fluids from Alzheimer's disease subjects. Amino Acids. 2007 Feb;32(2):213-24. Epub 2006 Oct 10. [PubMed ]
HMDB00641	L-Glutamine	Blood; CSF; Saliva; Urine	Fonteh AN, Harrington RJ, Tsai A, Liao P, Harrington MG: Free amino acid and dipeptide changes in the body fluids from Alzheimer's disease subjects. Amino Acids. 2007 Feb;32(2):213-24. Epub 2006 Oct 10. [PubMed ]
HMDB00177	L-Histidine	Blood; CSF; Saliva; Urine	Fonteh AN, Harrington RJ, Tsai A, Liao P, Harrington MG: Free amino acid and dipeptide changes in the body fluids from Alzheimer's disease subjects. Amino Acids. 2007 Feb;32(2):213-24. Epub 2006 Oct 10. [PubMed ]
HMDB00172	L-Isoleucine	Blood; CSF; Saliva; Urine	Fonteh AN, Harrington RJ, Tsai A, Liao P, Harrington MG: Free amino acid and dipeptide changes in the body fluids from Alzheimer's disease subjects. Amino Acids. 2007 Feb;32(2):213-24. Epub 2006 Oct 10. [PubMed ]
HMDB00687	L-Leucine	Blood; CSF; Urine	Fonteh AN, Harrington RJ, Tsai A, Liao P, Harrington MG: Free amino acid and dipeptide changes in the body fluids from Alzheimer's disease subjects. Amino Acids. 2007 Feb;32(2):213-24. Epub 2006 Oct 10. [PubMed ]
HMDB00182	L-Lysine	Blood; CSF; Saliva; Urine	Fonteh AN, Harrington RJ, Tsai A, Liao P, Harrington MG: Free amino acid and dipeptide changes in the body fluids from Alzheimer's disease subjects. Amino Acids. 2007 Feb;32(2):213-24. Epub 2006 Oct 10. [PubMed ]
HMDB00159	L-Phenylalanine	Blood; CSF; Saliva; Urine	Fonteh AN, Harrington RJ, Tsai A, Liao P, Harrington MG: Free amino acid and dipeptide changes in the body fluids from Alzheimer's disease subjects. Amino Acids. 2007 Feb;32(2):213-24. Epub 2006 Oct 10. [PubMed ]
HMDB00162	L-Proline	Blood; CSF; Saliva; Urine	Fonteh AN, Harrington RJ, Tsai A, Liao P, Harrington MG: Free amino acid and dipeptide changes in the body fluids from Alzheimer's disease subjects. Amino Acids. 2007 Feb;32(2):213-24. Epub 2006 Oct 10. [PubMed ]
HMDB00187	L-Serine	Blood; CSF; Urine	Fonteh AN, Harrington RJ, Tsai A, Liao P, Harrington MG: Free amino acid and dipeptide changes in the body fluids from Alzheimer's disease subjects. Amino Acids. 2007 Feb;32(2):213-24. Epub 2006 Oct 10. [PubMed ]
HMDB00167	L-Threonine	Blood; CSF; Saliva; Urine	Fonteh AN, Harrington RJ, Tsai A, Liao P, Harrington MG: Free amino acid and dipeptide changes in the body fluids from Alzheimer's disease subjects. Amino Acids. 2007 Feb;32(2):213-24. Epub 2006 Oct 10. [PubMed ]
HMDB00929	L-Tryptophan	Blood; CSF; Saliva; Urine	Fonteh AN, Harrington RJ, Tsai A, Liao P, Harrington MG: Free amino acid and dipeptide changes in the body fluids from Alzheimer's disease subjects. Amino Acids. 2007 Feb;32(2):213-24. Epub 2006 Oct 10. [PubMed ]
HMDB00158	L-Tyrosine	Blood; CSF; Saliva; Urine	Fonteh AN, Harrington RJ, Tsai A, Liao P, Harrington MG: Free amino acid and dipeptide changes in the body fluids from Alzheimer's disease subjects. Amino Acids. 2007 Feb;32(2):213-24. Epub 2006 Oct 10. [PubMed ]
HMDB00883	L-Valine	Blood; CSF; Saliva; Urine	Fonteh AN, Harrington RJ, Tsai A, Liao P, Harrington MG: Free amino acid and dipeptide changes in the body fluids from Alzheimer's disease subjects. Amino Acids. 2007 Feb;32(2):213-24. Epub 2006 Oct 10. [PubMed ]
HMDB04628	Lead	Blood; CSF; Urine	Bocca B, Forte G, Petrucci F, Pino A, Marchione F, Bomboi G, Senofonte O, Giubilei F, Alimonti A: Monitoring of chemical elements and oxidative damage in patients affected by Alzheimer's disease. Ann Ist Super Sanita. 2005;41(2):197-203. [PubMed ]
HMDB05949	Lithium	Blood	Bocca B, Forte G, Petrucci F, Pino A, Marchione F, Bomboi G, Senofonte O, Giubilei F, Alimonti A: Monitoring of chemical elements and oxidative damage in patients affected by Alzheimer's disease. Ann Ist Super Sanita. 2005;41(2):197-203. [PubMed ]
HMDB00547	Magnesium	Blood; CSF; Urine	Bocca B, Forte G, Petrucci F, Pino A, Marchione F, Bomboi G, Senofonte O, Giubilei F, Alimonti A: Monitoring of chemical elements and oxidative damage in patients affected by Alzheimer's disease. Ann Ist Super Sanita. 2005;41(2):197-203. [PubMed ]
HMDB01333	Manganese	Blood; CSF; Urine	Molina JA, Jimenez-Jimenez FJ, Aguilar MV, Meseguer I, Mateos-Vega CJ, Gonzalez-Munoz MJ, de Bustos F, Porta J, Orti-Pareja M, Zurdo M, Barrios E, Martinez-Para MC: Cerebrospinal fluid levels of transition metals in patients with Alzheimer's disease. J Neural Transm. 1998;105(4-5):479-88. [PubMed ]
HMDB00765	Mannitol	Blood; CSF; Urine	Shetty HU, Holloway HW, Schapiro MB: Cerebrospinal fluid and plasma distribution of myo-inositol and other polyols in Alzheimer disease. Clin Chem. 1996 Feb;42(2):298-302. [PubMed ]
HMDB03625	Mercury	Blood; CSF; Urine	Bocca B, Forte G, Petrucci F, Pino A, Marchione F, Bomboi G, Senofonte O, Giubilei F, Alimonti A: Monitoring of chemical elements and oxidative damage in patients affected by Alzheimer's disease. Ann Ist Super Sanita. 2005;41(2):197-203. [PubMed ]
HMDB00202	Methylmalonic acid	Blood; CSF; Urine	Kristensen MO, Gulmann NC, Christensen JE, Ostergaard K, Rasmussen K: Serum cobalamin and methylmalonic acid in Alzheimer dementia. Acta Neurol Scand. 1993 Jun;87(6):475-81. [PubMed ]
HMDB01302	Molybdenum	Blood; CSF; Urine	Bocca B, Forte G, Petrucci F, Pino A, Marchione F, Bomboi G, Senofonte O, Giubilei F, Alimonti A: Monitoring of chemical elements and oxidative damage in patients affected by Alzheimer's disease. Ann Ist Super Sanita. 2005;41(2):197-203. [PubMed ]
HMDB00211	Myoinositol	Blood; CSF; Urine	Shetty HU, Holloway HW, Schapiro MB: Cerebrospinal fluid and plasma distribution of myo-inositol and other polyols in Alzheimer disease. Clin Chem. 1996 Feb;42(2):298-302. [PubMed ]
HMDB02457	Nickel	Blood; CSF; Urine	Bocca B, Forte G, Petrucci F, Pino A, Marchione F, Bomboi G, Senofonte O, Giubilei F, Alimonti A: Monitoring of chemical elements and oxidative damage in patients affected by Alzheimer's disease. Ann Ist Super Sanita. 2005;41(2):197-203. [PubMed ]
HMDB00214	Ornithine	Blood; CSF; Saliva; Urine	Fonteh AN, Harrington RJ, Tsai A, Liao P, Harrington MG: Free amino acid and dipeptide changes in the body fluids from Alzheimer's disease subjects. Amino Acids. 2007 Feb;32(2):213-24. Epub 2006 Oct 10. [PubMed ]
HMDB03933	Pentosidine	Blood; CSF	Bar KJ, Franke S, Wenda B, Muller S, Kientsch-Engel R, Stein G, Sauer H: Pentosidine and N(epsilon)-(carboxymethyl)-lysine in Alzheimer's disease and vascular dementia. Neurobiol Aging. 2003 Mar-Apr;24(2):333-8. [PubMed ]
HMDB01565	Phosphorylcholine	Blood; Breast Milk; CSF	Walter A, Korth U, Hilgert M, Hartmann J, Weichel O, Hilgert M, Fassbender K, Schmitt A, Klein J: Glycerophosphocholine is elevated in cerebrospinal fluid of Alzheimer patients. Neurobiol Aging. 2004 Nov-Dec;25(10):1299-303. [PubMed ]
HMDB06695	Prolylhydroxyproline	CSF	Fonteh AN, Harrington RJ, Tsai A, Liao P, Harrington MG: Free amino acid and dipeptide changes in the body fluids from Alzheimer's disease subjects. Amino Acids. 2007 Feb;32(2):213-24. Epub 2006 Oct 10. [PubMed ]
HMDB00508	Ribitol	Blood; CSF; Urine	Shetty HU, Holloway HW, Schapiro MB: Cerebrospinal fluid and plasma distribution of myo-inositol and other polyols in Alzheimer disease. Clin Chem. 1996 Feb;42(2):298-302. [PubMed ]
HMDB02175	Silicon	Blood; CSF	Bocca B, Forte G, Petrucci F, Pino A, Marchione F, Bomboi G, Senofonte O, Giubilei F, Alimonti A: Monitoring of chemical elements and oxidative damage in patients affected by Alzheimer's disease. Ann Ist Super Sanita. 2005;41(2):197-203. [PubMed ]
HMDB00247	Sorbitol	Blood; CSF; Saliva; Urine	Shetty HU, Holloway HW, Schapiro MB: Cerebrospinal fluid and plasma distribution of myo-inositol and other polyols in Alzheimer disease. Clin Chem. 1996 Feb;42(2):298-302. [PubMed ]
HMDB03642	Strontium	Blood	Bocca B, Forte G, Petrucci F, Pino A, Marchione F, Bomboi G, Senofonte O, Giubilei F, Alimonti A: Monitoring of chemical elements and oxidative damage in patients affected by Alzheimer's disease. Ann Ist Super Sanita. 2005;41(2):197-203. [PubMed ]
HMDB00254	Succinic acid	Blood; CSF; Saliva; Urine	Redjems-Bennani N, Jeandel C, Lefebvre E, Blain H, Vidailhet M, Gueant JL: Abnormal substrate levels that depend upon mitochondrial function in cerebrospinal fluid from Alzheimer patients. Gerontology. 1998;44(5):300-4. [PubMed ]
HMDB02666	Thiamine monophosphate	Blood; CSF	Molina JA, Jimenez-Jimenez FJ, Hernanz A, Fernandez-Vivancos E, Medina S, de Bustos F, Gomez-Escalonilla C, Sayed Y: Cerebrospinal fluid levels of thiamine in patients with Alzheimer's disease. J Neural Transm. 2002 Jul;109(7-8):1035-44. [PubMed ]
HMDB01372	Thiamine pyrophosphate	Blood; CSF	Molina JA, Jimenez-Jimenez FJ, Hernanz A, Fernandez-Vivancos E, Medina S, de Bustos F, Gomez-Escalonilla C, Sayed Y: Cerebrospinal fluid levels of thiamine in patients with Alzheimer's disease. J Neural Transm. 2002 Jul;109(7-8):1035-44. [PubMed ]
HMDB01960	Tin	Blood; CSF	Bocca B, Forte G, Petrucci F, Pino A, Marchione F, Bomboi G, Senofonte O, Giubilei F, Alimonti A: Monitoring of chemical elements and oxidative damage in patients affected by Alzheimer's disease. Ann Ist Super Sanita. 2005;41(2):197-203. [PubMed ]
HMDB01966	Titanium	Blood	Bocca B, Forte G, Petrucci F, Pino A, Marchione F, Bomboi G, Senofonte O, Giubilei F, Alimonti A: Monitoring of chemical elements and oxidative damage in patients affected by Alzheimer's disease. Ann Ist Super Sanita. 2005;41(2):197-203. [PubMed ]
HMDB01989	Tungsten	Blood; CSF; Urine	Bocca B, Forte G, Petrucci F, Pino A, Marchione F, Bomboi G, Senofonte O, Giubilei F, Alimonti A: Monitoring of chemical elements and oxidative damage in patients affected by Alzheimer's disease. Ann Ist Super Sanita. 2005;41(2):197-203. [PubMed ]
HMDB02503	Vanadium	Blood; CSF	Bocca B, Forte G, Petrucci F, Pino A, Marchione F, Bomboi G, Senofonte O, Giubilei F, Alimonti A: Monitoring of chemical elements and oxidative damage in patients affected by Alzheimer's disease. Ann Ist Super Sanita. 2005;41(2):197-203. [PubMed ]
HMDB01303	Zinc	Blood; CSF; Urine	Bocca B, Forte G, Petrucci F, Pino A, Marchione F, Bomboi G, Senofonte O, Giubilei F, Alimonti A: Monitoring of chemical elements and oxidative damage in patients affected by Alzheimer's disease. Ann Ist Super Sanita. 2005;41(2):197-203. [PubMed ]

Ambulant pregnancy (3 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00153	Estriol	Amniotic Fluid; Blood; Urine	Andreolini F, Borra C, Caccamo F, Di Corcia A, Nicoletti I, Samperi R, Improta F: Estriol and its conjugates in late pregnancy determined by extraction with Carbopack B and liquid chromatography with fluorometric detection. Clin Chem. 1985 Oct;31(10):1698-702. [PubMed ]
HMDB10356	Estriol 3-sulfate 16-glucuronide	Amniotic Fluid; Blood; Urine	Andreolini F, Borra C, Caccamo F, Di Corcia A, Nicoletti I, Samperi R, Improta F: Estriol and its conjugates in late pregnancy determined by extraction with Carbopack B and liquid chromatography with fluorometric detection. Clin Chem. 1985 Oct;31(10):1698-702. [PubMed ]
HMDB10335	Estriol-3-glucuronide	Amniotic Fluid; Blood; Urine	Andreolini F, Borra C, Caccamo F, Di Corcia A, Nicoletti I, Samperi R, Improta F: Estriol and its conjugates in late pregnancy determined by extraction with Carbopack B and liquid chromatography with fluorometric detection. Clin Chem. 1985 Oct;31(10):1698-702. [PubMed ]

Aminoacylase I deficiency (2 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00532	Acetylglycine	Blood; Urine	Van Coster RN, Gerlo EA, Giardina TG, Engelke UF, Smet JE, De Praeter CM, Meersschaut VA, De Meirleir LJ, Seneca SH, Devreese B, Leroy JG, Herga S, Perrier JP, Wevers RA, Lissens W: Aminoacylase I deficiency: a novel inborn error of metabolism. Biochem Biophys Res Commun. 2005 Dec 23;338(3):1322-6. Epub 2005 Nov 2. [PubMed ] A J, Trygg J, Gullberg J, Johansson AI, Jonsson P, Antti H, Marklund SL, Moritz T: Extraction and GC/MS analysis of the human blood plasma metabolome. Anal Chem. 2005 Dec 15;77(24):8086-94. [PubMed ]
HMDB02931	N-Acetylserine	Urine	Sass JO, Mohr V, Olbrich H, Engelke U, Horvath J, Fliegauf M, Loges NT, Schweitzer-Krantz S, Moebus R, Weiler P, Kispert A, Superti-Furga A, Wevers RA, Omran H: Mutations in ACY1, the gene encoding aminoacylase 1, cause a novel inborn error of metabolism. Am J Hum Genet. 2006 Mar;78(3):401-9. Epub 2006 Jan 18. [PubMed ]

Amyotrophic lateral sclerosis (3 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00667	L-Thyronine	Blood; Urine	Malin JP, Kodding R, Fuhrmann H, von zur Muhlen A: T4, T3 and rT3 levels in serum and cerebrospinal fluid of patients with amyotrophic lateral sclerosis. J Neurol. 1989 Jan;236(1):57-9. [PubMed ]
HMDB02878	Nitrate	Blood; CSF	Boll MC, Alcaraz-Zubeldia M, Montes S, Murillo-Bonilla L, Rios C: Raised nitrate concentration and low SOD activity in the CSF of sporadic ALS patients. Neurochem Res. 2003 May;28(5):699-703. [PubMed ]
HMDB01220	Prostaglandin E2	Blood; CSF; Urine	Almer G, Teismann P, Stevic Z, Halaschek-Wiener J, Deecke L, Kostic V, Przedborski S: Increased levels of the pro-inflammatory prostaglandin PGE2 in CSF from ALS patients. Neurology. 2002 Apr 23;58(8):1277-9. [PubMed ]

Anemia (3 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00715	Kynurenic acid	Blood; CSF; Urine	Medana IM, Day NP, Salahifar-Sabet H, Stocker R, Smythe G, Bwanaisa L, Njobvu A, Kayira K, Turner GD, Taylor TE, Hunt NH: Metabolites of the kynurenine pathway of tryptophan metabolism in the cerebrospinal fluid of Malawian children with malaria. J Infect Dis. 2003 Sep 15;188(6):844-9. Epub 2003 Sep 9. [PubMed ]
HMDB00070	Pipecolic acid	Amniotic Fluid; Blood; CSF; Urine	Medana IM, Day NP, Salahifar-Sabet H, Stocker R, Smythe G, Bwanaisa L, Njobvu A, Kayira K, Turner GD, Taylor TE, Hunt NH: Metabolites of the kynurenine pathway of tryptophan metabolism in the cerebrospinal fluid of Malawian children with malaria. J Infect Dis. 2003 Sep 15;188(6):844-9. Epub 2003 Sep 9. [PubMed ]
HMDB00232	Quinolinic acid	Blood; CSF; Urine	Medana IM, Day NP, Salahifar-Sabet H, Stocker R, Smythe G, Bwanaisa L, Njobvu A, Kayira K, Turner GD, Taylor TE, Hunt NH: Metabolites of the kynurenine pathway of tryptophan metabolism in the cerebrospinal fluid of Malawian children with malaria. J Infect Dis. 2003 Sep 15;188(6):844-9. Epub 2003 Sep 9. [PubMed ]

Anephrism (6 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00430	24,25-Dihydroxyvitamin D	Blood	Shepard RM, Horst RL, Hamstra AJ, DeLuca HF: Determination of vitamin D and its metabolites in plasma from normal and anephric man. Biochem J. 1979 Jul 15;182(1):55-69. [PubMed ]
HMDB01420	25,26-dihydroxyvitamin D	Blood	Shepard RM, Horst RL, Hamstra AJ, DeLuca HF: Determination of vitamin D and its metabolites in plasma from normal and anephric man. Biochem J. 1979 Jul 15;182(1):55-69. [PubMed ]
HMDB01438	25-Hydroxyvitamin D2	Blood; Urine	Shepard RM, Horst RL, Hamstra AJ, DeLuca HF: Determination of vitamin D and its metabolites in plasma from normal and anephric man. Biochem J. 1979 Jul 15;182(1):55-69. [PubMed ]
HMDB03550	Calcidiol	Blood; Urine	Shepard RM, Horst RL, Hamstra AJ, DeLuca HF: Determination of vitamin D and its metabolites in plasma from normal and anephric man. Biochem J. 1979 Jul 15;182(1):55-69. [PubMed ]
HMDB00900	Ergocalciferol	Blood	Shepard RM, Horst RL, Hamstra AJ, DeLuca HF: Determination of vitamin D and its metabolites in plasma from normal and anephric man. Biochem J. 1979 Jul 15;182(1):55-69. [PubMed ]
HMDB00876	Vitamin D3	Blood	Shepard RM, Horst RL, Hamstra AJ, DeLuca HF: Determination of vitamin D and its metabolites in plasma from normal and anephric man. Biochem J. 1979 Jul 15;182(1):55-69. [PubMed ]

Angina (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB04242	11-Dehydro-thromboxane B2	Blood; Urine	Koike T, Hattori A, Igarashi Y, Matsubara T, Tamura Y, Yamazoe M, Izumi T, Shibata A: [Elevation of 11-dehydro-thromboxane B2 levels in unstable angina] J Cardiol. 1991;21(4):899-904. [PubMed ]

Anorexia nervosa (13 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00526	5a-Tetrahydrocortisol	Blood; Urine	Vierhapper H, Kiss A, Nowotny P, Wiesnagrotzki S, Monder C, Waldhausl W: Metabolism of cortisol in anorexia nervosa. Acta Endocrinol (Copenh). 1990 Jun;122(6):753-8. [PubMed ]
HMDB00448	Adipic acid	Blood; CSF; Urine	Capo-chichi CD, Gueant JL, Lefebvre E, Bennani N, Lorentz E, Vidailhet C, Vidailhet M: Riboflavin and riboflavin-derived cofactors in adolescent girls with anorexia nervosa. Am J Clin Nutr. 1999 Apr;69(4):672-8. [PubMed ]
HMDB00063	Cortisol	Blood; CSF; Saliva; Urine	Vierhapper H, Kiss A, Nowotny P, Wiesnagrotzki S, Monder C, Waldhausl W: Metabolism of cortisol in anorexia nervosa. Acta Endocrinol (Copenh). 1990 Jun;122(6):753-8. [PubMed ]
HMDB00622	Ethylmalonic acid	CSF; Urine	Capo-chichi CD, Gueant JL, Lefebvre E, Bennani N, Lorentz E, Vidailhet C, Vidailhet M: Riboflavin and riboflavin-derived cofactors in adolescent girls with anorexia nervosa. Am J Clin Nutr. 1999 Apr;69(4):672-8. [PubMed ]
HMDB01248	FAD	Blood	Capo-chichi CD, Gueant JL, Lefebvre E, Bennani N, Lorentz E, Vidailhet C, Vidailhet M: Riboflavin and riboflavin-derived cofactors in adolescent girls with anorexia nervosa. Am J Clin Nutr. 1999 Apr;69(4):672-8. [PubMed ]
HMDB01520	Flavin Mononucleotide	Blood	Capo-chichi CD, Gueant JL, Lefebvre E, Bennani N, Lorentz E, Vidailhet C, Vidailhet M: Riboflavin and riboflavin-derived cofactors in adolescent girls with anorexia nervosa. Am J Clin Nutr. 1999 Apr;69(4):672-8. [PubMed ]
HMDB00661	Glutaric acid	Blood; Urine	Capo-chichi CD, Gueant JL, Lefebvre E, Bennani N, Lorentz E, Vidailhet C, Vidailhet M: Riboflavin and riboflavin-derived cofactors in adolescent girls with anorexia nervosa. Am J Clin Nutr. 1999 Apr;69(4):672-8. [PubMed ]
HMDB00678	Isovalerylglycine	Blood; Urine	Capo-chichi CD, Gueant JL, Lefebvre E, Bennani N, Lorentz E, Vidailhet C, Vidailhet M: Riboflavin and riboflavin-derived cofactors in adolescent girls with anorexia nervosa. Am J Clin Nutr. 1999 Apr;69(4):672-8. [PubMed ]
HMDB00265	Liothyronine	Blood; Saliva; Urine	Capo-chichi CD, Gueant JL, Lefebvre E, Bennani N, Lorentz E, Vidailhet C, Vidailhet M: Riboflavin and riboflavin-derived cofactors in adolescent girls with anorexia nervosa. Am J Clin Nutr. 1999 Apr;69(4):672-8. [PubMed ]
HMDB00244	Riboflavin	Blood; CSF; Urine	Capo-chichi CD, Gueant JL, Lefebvre E, Bennani N, Lorentz E, Vidailhet C, Vidailhet M: Riboflavin and riboflavin-derived cofactors in adolescent girls with anorexia nervosa. Am J Clin Nutr. 1999 Apr;69(4):672-8. [PubMed ]
HMDB00893	Suberic acid	Blood; CSF; Urine	Capo-chichi CD, Gueant JL, Lefebvre E, Bennani N, Lorentz E, Vidailhet C, Vidailhet M: Riboflavin and riboflavin-derived cofactors in adolescent girls with anorexia nervosa. Am J Clin Nutr. 1999 Apr;69(4):672-8. [PubMed ]
HMDB00903	Tetrahydrocortisone	Blood; Urine	Vierhapper H, Kiss A, Nowotny P, Wiesnagrotzki S, Monder C, Waldhausl W: Metabolism of cortisol in anorexia nervosa. Acta Endocrinol (Copenh). 1990 Jun;122(6):753-8. [PubMed ]
HMDB00248	Thyroxine	Blood; Saliva; Urine	Capo-chichi CD, Gueant JL, Lefebvre E, Bennani N, Lorentz E, Vidailhet C, Vidailhet M: Riboflavin and riboflavin-derived cofactors in adolescent girls with anorexia nervosa. Am J Clin Nutr. 1999 Apr;69(4):672-8. [PubMed ]

Anoxia (9 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00357	3-Hydroxybutyric acid	Blood; Cellular Cytoplasm; CSF; Urine	Zupke C, Sinskey AJ, Stephanopoulos G: Intracellular flux analysis applied to the effect of dissolved oxygen on hybridomas. Appl Microbiol Biotechnol. 1995 Dec;44(1-2):27-36. [PubMed ]
HMDB00060	Acetoacetic acid	Blood; Cellular Cytoplasm; CSF; Urine	Zupke C, Sinskey AJ, Stephanopoulos G: Intracellular flux analysis applied to the effect of dissolved oxygen on hybridomas. Appl Microbiol Biotechnol. 1995 Dec;44(1-2):27-36. [PubMed ]
HMDB00193	Isocitric acid	Blood; Cellular Cytoplasm; CSF; Semen; Urine	Zupke C, Sinskey AJ, Stephanopoulos G: Intracellular flux analysis applied to the effect of dissolved oxygen on hybridomas. Appl Microbiol Biotechnol. 1995 Dec;44(1-2):27-36. [PubMed ]
HMDB00148	L-Glutamic acid	Blood; Cellular Cytoplasm; CSF; Saliva; Urine	Zupke C, Sinskey AJ, Stephanopoulos G: Intracellular flux analysis applied to the effect of dissolved oxygen on hybridomas. Appl Microbiol Biotechnol. 1995 Dec;44(1-2):27-36. [PubMed ]
HMDB00190	L-Lactic acid	Bile; Blood; Cellular Cytoplasm; CSF; Urine	Zupke C, Sinskey AJ, Stephanopoulos G: Intracellular flux analysis applied to the effect of dissolved oxygen on hybridomas. Appl Microbiol Biotechnol. 1995 Dec;44(1-2):27-36. [PubMed ]
HMDB00156	L-Malic acid	Blood; Cellular Cytoplasm; CSF; Urine	Zupke C, Sinskey AJ, Stephanopoulos G: Intracellular flux analysis applied to the effect of dissolved oxygen on hybridomas. Appl Microbiol Biotechnol. 1995 Dec;44(1-2):27-36. [PubMed ]
HMDB00223	Oxalacetic acid	Cellular Cytoplasm; CSF; Urine	Zupke C, Sinskey AJ, Stephanopoulos G: Intracellular flux analysis applied to the effect of dissolved oxygen on hybridomas. Appl Microbiol Biotechnol. 1995 Dec;44(1-2):27-36. [PubMed ]
HMDB00208	Oxoglutaric acid	Blood; Cellular Cytoplasm; CSF; N/A; Urine	Zupke C, Sinskey AJ, Stephanopoulos G: Intracellular flux analysis applied to the effect of dissolved oxygen on hybridomas. Appl Microbiol Biotechnol. 1995 Dec;44(1-2):27-36. [PubMed ]
HMDB00243	Pyruvic acid	Blood; Cellular Cytoplasm; CSF; Saliva; Urine	Zupke C, Sinskey AJ, Stephanopoulos G: Intracellular flux analysis applied to the effect of dissolved oxygen on hybridomas. Appl Microbiol Biotechnol. 1995 Dec;44(1-2):27-36. [PubMed ]

Appendicitis (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00197	Indoleacetic acid	Blood; CSF; Urine	Ilkhanizadeh B, Owji AA, Tavangar SM, Vasei M, Tabei SM: Spot urine 5-hydroxy indole acetic acid and acute appendicitis. Hepatogastroenterology. 2001 May-Jun;48(39):609-13. [PubMed ]

Argininemia (3 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00052	Argininosuccinic acid	Blood; Urine	Metagene: ARGININEMIA. HYPERARGININEMIA, ARGINASE DEFICIENCY
HMDB00517	L-Arginine	Blood; CSF; Urine	Metagene: ARGININEMIA. HYPERARGININEMIA, ARGINASE DEFICIENCY
HMDB00300	Uracil	Amniotic Fluid; Blood; CSF; Urine	Metagene: ARGININEMIA. HYPERARGININEMIA, ARGINASE DEFICIENCY

Argininosuccinic aciduria (ASL) (2 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00051	Ammonia	Blood; Cellular Cytoplasm; CSF; Urine	Metagene: ARGININOSUCCINIC ACIDURIA (ASL)
HMDB00904	Citrulline	Blood; CSF; Urine	Metagene: ARGININOSUCCINIC ACIDURIA (ASL)

Argininosuccinicaciduria (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00052	Argininosuccinic acid	Blood; Urine	Lee CR, Pollitt RJ: New derivatives of argininosuccinic acid in the urine of a patient with argininosuccinicaciduria. Biochem J. 1972 Jan;126(1):79-87. [PubMed ]

Aromatic L-amino acid decarboxylase deficiency (9 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB01434	3-Methoxytyrosine	Blood; CSF; Urine	Abdenur JE, Abeling N, Specola N, Jorge L, Schenone AB, van Cruchten AC, Chamoles NA: Aromatic l-aminoacid decarboxylase deficiency: unusual neonatal presentation and additional findings in organic acid analysis. Mol Genet Metab. 2006 Jan;87(1):48-53. Epub 2005 Nov 9. [PubMed ]
HMDB00763	5-Hydroxyindoleacetic acid	Blood; CSF; Urine	Abdenur JE, Abeling N, Specola N, Jorge L, Schenone AB, van Cruchten AC, Chamoles NA: Aromatic l-aminoacid decarboxylase deficiency: unusual neonatal presentation and additional findings in organic acid analysis. Mol Genet Metab. 2006 Jan;87(1):48-53. Epub 2005 Nov 9. [PubMed ]
HMDB00073	Dopamine	Blood; CSF; Urine	Abdenur JE, Abeling N, Specola N, Jorge L, Schenone AB, van Cruchten AC, Chamoles NA: Aromatic l-aminoacid decarboxylase deficiency: unusual neonatal presentation and additional findings in organic acid analysis. Mol Genet Metab. 2006 Jan;87(1):48-53. Epub 2005 Nov 9. [PubMed ]
HMDB00118	Homovanillic acid	Blood; CSF; Urine	Abdenur JE, Abeling N, Specola N, Jorge L, Schenone AB, van Cruchten AC, Chamoles NA: Aromatic l-aminoacid decarboxylase deficiency: unusual neonatal presentation and additional findings in organic acid analysis. Mol Genet Metab. 2006 Jan;87(1):48-53. Epub 2005 Nov 9. [PubMed ]
HMDB00866	N-Acetyl-L-tyrosine	Blood; Urine	Abdenur JE, Abeling N, Specola N, Jorge L, Schenone AB, van Cruchten AC, Chamoles NA: Aromatic l-aminoacid decarboxylase deficiency: unusual neonatal presentation and additional findings in organic acid analysis. Mol Genet Metab. 2006 Jan;87(1):48-53. Epub 2005 Nov 9. [PubMed ]
HMDB11716	N-Acetylvanilalanine	Urine	Abdenur JE, Abeling N, Specola N, Jorge L, Schenone AB, van Cruchten AC, Chamoles NA: Aromatic l-aminoacid decarboxylase deficiency: unusual neonatal presentation and additional findings in organic acid analysis. Mol Genet Metab. 2006 Jan;87(1):48-53. Epub 2005 Nov 9. [PubMed ]
HMDB00291	Vanillylmandelic acid	Blood; Urine	Abdenur JE, Abeling N, Specola N, Jorge L, Schenone AB, van Cruchten AC, Chamoles NA: Aromatic l-aminoacid decarboxylase deficiency: unusual neonatal presentation and additional findings in organic acid analysis. Mol Genet Metab. 2006 Jan;87(1):48-53. Epub 2005 Nov 9. [PubMed ]
HMDB11714	Vanilpyruvic acid	Blood; Urine	Abdenur JE, Abeling N, Specola N, Jorge L, Schenone AB, van Cruchten AC, Chamoles NA: Aromatic l-aminoacid decarboxylase deficiency: unusual neonatal presentation and additional findings in organic acid analysis. Mol Genet Metab. 2006 Jan;87(1):48-53. Epub 2005 Nov 9. [PubMed ]
HMDB01490	Vanylglycol	Blood; CSF; Urine	Abdenur JE, Abeling N, Specola N, Jorge L, Schenone AB, van Cruchten AC, Chamoles NA: Aromatic l-aminoacid decarboxylase deficiency: unusual neonatal presentation and additional findings in organic acid analysis. Mol Genet Metab. 2006 Jan;87(1):48-53. Epub 2005 Nov 9. [PubMed ]

Arsenic trioxide treatment for acute promyelocytic leukemia (APL) (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB11620	Arsenite	Blood; CSF	Au WY, Tam S, Fong BM, Kwong YL: Determinants of cerebrospinal fluid arsenic concentration in patients with acute promyelocytic leukemia on oral arsenic trioxide therapy. Blood. 2008 Nov 1;112(9):3587-90. Epub 2008 Aug 14. [PubMed ]

Arteriosclerosis (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB03584	Taurocyamine	Blood	Kai M, Miyazaki T, Ohkura Y: High-performance liquid chromatographic measurement of guanidino compounds of clinical importance in human urine and serum by pre-column fluorescence derivatization using benzoin. J Chromatogr. 1984 Nov 28;311(2):257-66. [PubMed ]

Aspartylglucosaminuria (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00489	Aspartylglycosamine	Urine	Metagene: ASPARTYLGLUCOSAMINURIA

Asthma (13 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB02123	1,3,7-Trimethyluric acid	Urine	Zydron M, Baranowski J, Baranowska I: Separation, pre-concentration, and HPLC analysis of methylxanthines in urine samples. J Sep Sci. 2004 Oct;27(14):1166-72. [PubMed ]
HMDB01857	1,3-Dimethyluric acid	Urine	Zydron M, Baranowski J, Baranowska I: Separation, pre-concentration, and HPLC analysis of methylxanthines in urine samples. J Sep Sci. 2004 Oct;27(14):1166-72. [PubMed ]
HMDB11103	1,7-Dimethyluric acid	Urine	Zydron M, Baranowski J, Baranowska I: Separation, pre-concentration, and HPLC analysis of methylxanthines in urine samples. J Sep Sci. 2004 Oct;27(14):1166-72. [PubMed ]
HMDB10738	1-Methylxanthine	Urine	Zydron M, Baranowski J, Baranowska I: Separation, pre-concentration, and HPLC analysis of methylxanthines in urine samples. J Sep Sci. 2004 Oct;27(14):1166-72. [PubMed ]
HMDB10199	11b-PGF2a	Blood; Urine	Mai XM, Bottcher MF, Bruhammar M, Nilsson L, Zetterstrom O: Urinary inflammatory mediators and inhalation of hypertonic saline in children. Allergy. 2005 Jan;60(1):60-4. [PubMed ]
HMDB01885	3-Chlorotyrosine	Blood; Urine	Mita H, Higashi N, Taniguchi M, Higashi A, Kawagishi Y, Akiyama K: Urinary 3-bromotyrosine and 3-chlorotyrosine concentrations in asthmatic patients: lack of increase in 3-bromotyrosine concentration in urine and plasma proteins in aspirin-induced asthma after intravenous aspirin challenge. Clin Exp Allergy. 2004 Jun;34(6):931-8. [PubMed ]
HMDB01886	3-Methylxanthine	Urine	Zydron M, Baranowski J, Baranowska I: Separation, pre-concentration, and HPLC analysis of methylxanthines in urine samples. J Sep Sci. 2004 Oct;27(14):1166-72. [PubMed ]
HMDB11134	5-HETE	Blood	Chavis C, Fraissinet L, Chanez P, Thomas E, Bousquet J: A method for the measurement of plasma hydroxyeicosatetraenoic acid levels. Anal Biochem. 1999 Jun 15;271(1):105-8. [PubMed ]
HMDB01991	7-Methylxanthine	Urine	Zydron M, Baranowski J, Baranowska I: Separation, pre-concentration, and HPLC analysis of methylxanthines in urine samples. J Sep Sci. 2004 Oct;27(14):1166-72. [PubMed ]
HMDB01847	Caffeine	Blood; Urine	Zydron M, Baranowski J, Baranowska I: Separation, pre-concentration, and HPLC analysis of methylxanthines in urine samples. J Sep Sci. 2004 Oct;27(14):1166-72. [PubMed ]
HMDB01860	Paraxanthine	Blood; Urine	Zydron M, Baranowski J, Baranowska I: Separation, pre-concentration, and HPLC analysis of methylxanthines in urine samples. J Sep Sci. 2004 Oct;27(14):1166-72. [PubMed ]
HMDB02825	Theobromine	Blood; Urine	Zydron M, Baranowski J, Baranowska I: Separation, pre-concentration, and HPLC analysis of methylxanthines in urine samples. J Sep Sci. 2004 Oct;27(14):1166-72. [PubMed ]
HMDB01889	Theophylline	Blood; Urine	Zydron M, Baranowski J, Baranowska I: Separation, pre-concentration, and HPLC analysis of methylxanthines in urine samples. J Sep Sci. 2004 Oct;27(14):1166-72. [PubMed ]

Athyroic patients (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00021	Iodotyrosine	Blood	Tan SA, Lewis JE, Berk LS, Wilcox RB: Extrathyroidal physiology of monoiodotyrosine in humans. Clin Physiol Biochem. 1990;8(3):109-15. [PubMed ]

Autism (2 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00118	Homovanillic acid	Blood; CSF; Urine	Narayan M, Srinath S, Anderson GM, Meundi DB: Cerebrospinal fluid levels of homovanillic acid and 5-hydroxyindoleacetic acid in autism. Biol Psychiatry. 1993 Apr 15-May 1;33(8-9):630-5. [PubMed ]
HMDB00099	L-Cystathionine	Blood; CSF; Urine	James SJ, Cutler P, Melnyk S, Jernigan S, Janak L, Gaylor DW, Neubrander JA: Metabolic biomarkers of increased oxidative stress and impaired methylation capacity in children with autism. Am J Clin Nutr. 2004 Dec;80(6):1611-7. [PubMed ]

Autosomal dominant polycystic kidney disease (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB01539	Dimethyl-L-arginine	Blood; CSF; Urine	Wang D, Strandgaard S, Borresen ML, Luo Z, Connors SG, Yan Q, Wilcox CS: Asymmetric dimethylarginine and lipid peroxidation products in early autosomal dominant polycystic kidney disease. Am J Kidney Dis. 2008 Feb;51(2):184-91. [PubMed ]

Autosomal dominant polycystic kidney disease (ADPKD) (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB04667	13S-hydroxyoctadecadienoic acid	Blood; Urine	Wang D, Strandgaard S, Borresen ML, Luo Z, Connors SG, Yan Q, Wilcox CS: Asymmetric dimethylarginine and lipid peroxidation products in early autosomal dominant polycystic kidney disease. Am J Kidney Dis. 2008 Feb;51(2):184-91. [PubMed ]

Autosomal dominant polycystic kidney diseases (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB03334	Symmetric dimethylarginine	Blood; Urine	Wang D, Strandgaard S, Borresen ML, Luo Z, Connors SG, Yan Q, Wilcox CS: Asymmetric dimethylarginine and lipid peroxidation products in early autosomal dominant polycystic kidney disease. Am J Kidney Dis. 2008 Feb;51(2):184-91. [PubMed ]

Bacterial infections (assorted) (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00159	L-Phenylalanine	Blood; CSF; Saliva; Urine	Wannemacher RW Jr, Klainer AS, Dinterman RE, Beisel WR: The significance and mechanism of an increased serum phenylalanine-tyrosine ratio during infection. Am J Clin Nutr. 1976 Sep;29(9):997-1006. [PubMed ]

Benign prostatic hypertrophy (BpH) (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00495	Androstanediol	Urine	Puah CM, Williams G, Ghanadian R: Urinary unconjugated 5 alpha-androstane-3 alpha, 17 beta-diol in patients with prostatic tumours. Urol Res. 1982;10(2):81-4. [PubMed ]

Benzene exposure (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB02349	trans-trans-Muconic acid	Urine	Chanvaivit S, Navasumrit P, Hunsonti P, Autrup H, Ruchirawat M: Exposure assessment of benzene in Thai workers, DNA-repair capacity and influence of genetic polymorphisms. Mutat Res. 2007 Jan 10;626(1-2):79-87. Epub 2006 Nov 13. [PubMed ]

Beta-Ketothiolase deficiency (3 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00354	2-Methyl-3-hydroxybutyric acid	Urine	Metagene: BETA-KETOTHIOLASE DEFICIENCY
HMDB03771	2-Methylacetoacetic acid	Urine	Metagene: BETA-KETOTHIOLASE DEFICIENCY
HMDB00959	Tiglylglycine	Urine	Metagene: BETA-KETOTHIOLASE DEFICIENCY

Beta-thalassemia (2 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB10316	Acetaminophen glucuronide	Blood	Tankanitlert J, Howard TA, Temsakulphong A, Sirankapracha P, Morales NP, Sanvarinda Y, Fucharoen P, Ware RE, Fucharoen S, Chantharaksri U: A pharmacokinetic study of paracetamol in Thai beta-thalassemia/HbE patients. Eur J Clin Pharmacol. 2006 Sep;62(9):743-8. Epub 2006 Jul 15. [PubMed ]
HMDB01060	Ubiquinol 8	Blood	De Luca C, Filosa A, Grandinetti M, Maggio F, Lamba M, Passi S: Blood antioxidant status and urinary levels of catecholamine metabolites in beta-thalassemia. Free Radic Res. 1999 Jun;30(6):453-62. [PubMed ]

Beta-ureidopropionase deficiency (3 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00079	Dihydrothymine	CSF; Urine	van Kuilenburg AB, Meinsma R, Beke E, Assmann B, Ribes A, Lorente I, Busch R, Mayatepek E, Abeling NG, van Cruchten A, Stroomer AE, van Lenthe H, Zoetekouw L, Kulik W, Hoffmann GF, Voit T, Wevers RA, Rutsch F, van Gennip AH: beta-Ureidopropionase deficiency: an inborn error of pyrimidine degradation associated with neurological abnormalities. Hum Mol Genet. 2004 Nov 15;13(22):2793-801. Epub 2004 Sep 22. [PubMed ]
HMDB00262	Thymine	Blood; CSF; Urine	van Kuilenburg AB, Meinsma R, Beke E, Assmann B, Ribes A, Lorente I, Busch R, Mayatepek E, Abeling NG, van Cruchten A, Stroomer AE, van Lenthe H, Zoetekouw L, Kulik W, Hoffmann GF, Voit T, Wevers RA, Rutsch F, van Gennip AH: beta-Ureidopropionase deficiency: an inborn error of pyrimidine degradation associated with neurological abnormalities. Hum Mol Genet. 2004 Nov 15;13(22):2793-801. Epub 2004 Sep 22. [PubMed ]
HMDB02031	Ureidoisobutyric acid	CSF; Urine	van Kuilenburg AB, Meinsma R, Beke E, Assmann B, Ribes A, Lorente I, Busch R, Mayatepek E, Abeling NG, van Cruchten A, Stroomer AE, van Lenthe H, Zoetekouw L, Kulik W, Hoffmann GF, Voit T, Wevers RA, Rutsch F, van Gennip AH: beta-Ureidopropionase deficiency: an inborn error of pyrimidine degradation associated with neurological abnormalities. Hum Mol Genet. 2004 Nov 15;13(22):2793-801. Epub 2004 Sep 22. [PubMed ]

Bilateral testicular adrenal rest tumors (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB04030	21-Deoxycortisol	Blood	Claahsen-van der Grinten HL, Otten BJ, Sweep FC, Span PN, Ross HA, Meuleman EJ, Hermus AR: Testicular tumors in patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency show functional features of adrenocortical tissue. J Clin Endocrinol Metab. 2007 Sep;92(9):3674-80. Epub 2007 Jun 26. [PubMed ]

Biliary atresia (5 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00518	Chenodeoxycholic acid	Bile; Blood; Urine	Nittono H, Obinata K, Nakatsu N, Watanabe T, Niijima S, Sasaki H, Arisaka O, Kato H, Yabuta K, Miyano T: Sulfated and nonsulfated bile acids in urine of patients with biliary atresia: analysis of bile acids by high-performance liquid chromatography. J Pediatr Gastroenterol Nutr. 1986 Jan;5(1):23-9. [PubMed ]
HMDB00637	Chenodeoxycholic acid glycine conjugate	Bile; Blood; Urine	Nittono H, Obinata K, Nakatsu N, Watanabe T, Niijima S, Sasaki H, Arisaka O, Kato H, Yabuta K, Miyano T: Sulfated and nonsulfated bile acids in urine of patients with biliary atresia: analysis of bile acids by high-performance liquid chromatography. J Pediatr Gastroenterol Nutr. 1986 Jan;5(1):23-9. [PubMed ]
HMDB02061	Hyaluronic acid	Blood; CSF	Ukarapol N, Wongsawasdi L, Ong-Chai S, Riddhiputra P, Kongtawelert P: Hyaluronic acid: additional biochemical marker in the diagnosis of biliary atresia. Pediatr Int. 2007 Oct;49(5):608-11. [PubMed ]
HMDB00951	Taurochenodesoxycholic acid	Bile; Blood; Urine	Nittono H, Obinata K, Nakatsu N, Watanabe T, Niijima S, Sasaki H, Arisaka O, Kato H, Yabuta K, Miyano T: Sulfated and nonsulfated bile acids in urine of patients with biliary atresia: analysis of bile acids by high-performance liquid chromatography. J Pediatr Gastroenterol Nutr. 1986 Jan;5(1):23-9. [PubMed ]
HMDB00036	Taurocholic acid	Bile; Blood; Urine	Nittono H, Obinata K, Nakatsu N, Watanabe T, Niijima S, Sasaki H, Arisaka O, Kato H, Yabuta K, Miyano T: Sulfated and nonsulfated bile acids in urine of patients with biliary atresia: analysis of bile acids by high-performance liquid chromatography. J Pediatr Gastroenterol Nutr. 1986 Jan;5(1):23-9. [PubMed ]

Biliary cirrhosis (10 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00326	1b,3a,12a-Trihydroxy-5b-cholanoic acid	Blood; Urine	Batta AK, Arora R, Salen G, Tint GS, Eskreis D, Katz S: Characterization of serum and urinary bile acids in patients with primary biliary cirrhosis by gas-liquid chromatography-mass spectrometry: effect of ursodeoxycholic acid treatment. J Lipid Res. 1989 Dec;30(12):1953-62. [PubMed ]
HMDB00518	Chenodeoxycholic acid	Bile; Blood; Urine	Batta AK, Arora R, Salen G, Tint GS, Eskreis D, Katz S: Characterization of serum and urinary bile acids in patients with primary biliary cirrhosis by gas-liquid chromatography-mass spectrometry: effect of ursodeoxycholic acid treatment. J Lipid Res. 1989 Dec;30(12):1953-62. [PubMed ]
HMDB00619	Cholic acid	Bile; Blood; Urine	Batta AK, Arora R, Salen G, Tint GS, Eskreis D, Katz S: Characterization of serum and urinary bile acids in patients with primary biliary cirrhosis by gas-liquid chromatography-mass spectrometry: effect of ursodeoxycholic acid treatment. J Lipid Res. 1989 Dec;30(12):1953-62. [PubMed ]
HMDB00626	Deoxycholic acid	Bile; Blood; Urine	Batta AK, Arora R, Salen G, Tint GS, Eskreis D, Katz S: Characterization of serum and urinary bile acids in patients with primary biliary cirrhosis by gas-liquid chromatography-mass spectrometry: effect of ursodeoxycholic acid treatment. J Lipid Res. 1989 Dec;30(12):1953-62. [PubMed ]
HMDB00760	Hyocholic acid	Blood; Urine	Batta AK, Arora R, Salen G, Tint GS, Eskreis D, Katz S: Characterization of serum and urinary bile acids in patients with primary biliary cirrhosis by gas-liquid chromatography-mass spectrometry: effect of ursodeoxycholic acid treatment. J Lipid Res. 1989 Dec;30(12):1953-62. [PubMed ]
HMDB00733	Hyodeoxycholic acid	Blood; Urine	Batta AK, Arora R, Salen G, Tint GS, Eskreis D, Katz S: Characterization of serum and urinary bile acids in patients with primary biliary cirrhosis by gas-liquid chromatography-mass spectrometry: effect of ursodeoxycholic acid treatment. J Lipid Res. 1989 Dec;30(12):1953-62. [PubMed ]
HMDB00686	Isoursodeoxycholic acid	Blood; Urine	Batta AK, Arora R, Salen G, Tint GS, Eskreis D, Katz S: Characterization of serum and urinary bile acids in patients with primary biliary cirrhosis by gas-liquid chromatography-mass spectrometry: effect of ursodeoxycholic acid treatment. J Lipid Res. 1989 Dec;30(12):1953-62. [PubMed ]
HMDB00761	Lithocholic acid	Bile; Blood; Urine	Batta AK, Arora R, Salen G, Tint GS, Eskreis D, Katz S: Characterization of serum and urinary bile acids in patients with primary biliary cirrhosis by gas-liquid chromatography-mass spectrometry: effect of ursodeoxycholic acid treatment. J Lipid Res. 1989 Dec;30(12):1953-62. [PubMed ]
HMDB00917	Ursocholic acid	Blood; Urine	Batta AK, Arora R, Salen G, Tint GS, Eskreis D, Katz S: Characterization of serum and urinary bile acids in patients with primary biliary cirrhosis by gas-liquid chromatography-mass spectrometry: effect of ursodeoxycholic acid treatment. J Lipid Res. 1989 Dec;30(12):1953-62. [PubMed ]
HMDB00946	Ursodeoxycholic acid	Blood; Urine	Batta AK, Arora R, Salen G, Tint GS, Eskreis D, Katz S: Characterization of serum and urinary bile acids in patients with primary biliary cirrhosis by gas-liquid chromatography-mass spectrometry: effect of ursodeoxycholic acid treatment. J Lipid Res. 1989 Dec;30(12):1953-62. [PubMed ]

Biotinidase deficiency (4 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00379	2-Methylcitric acid	Blood; CSF; Urine	Metagene: BIOTINIDASE DEFICIENCY
HMDB00754	3-Hydroxyisovaleric acid	Blood; CSF; Urine	Metagene: BIOTINIDASE DEFICIENCY
HMDB00459	3-Methylcrotonylglycine	Urine	Metagene: BIOTINIDASE DEFICIENCY
HMDB00700	Hydroxypropionic acid	Blood; CSF; Urine	Metagene: BIOTINIDASE DEFICIENCY

Bladder infections (2 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB02039	2-Pyrrolidinone	Blood	Tricker AR, Pfundstein B, Kalble T, Preussmann R: Secondary amine precursors to nitrosamines in human saliva, gastric juice, blood, urine and faeces. Carcinogenesis. 1992 Apr;13(4):563-8. [PubMed ]
HMDB00087	Dimethylamine	Blood; CSF; Saliva; Urine	Tricker AR, Pfundstein B, Kalble T, Preussmann R: Secondary amine precursors to nitrosamines in human saliva, gastric juice, blood, urine and faeces. Carcinogenesis. 1992 Apr;13(4):563-8. [PubMed ]

Bone metastases (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00452	L-Alpha-aminobutyric acid	Blood; CSF; Urine	Tsuruta Y, Matsumoto M, Inoue H, Munemura S, Yamano S, Iguchi H: Determination of N-ethylglycine in urine of cancer patients with metastatic bone disease by HPLC using 4-(5,6-dimethoxy-2-phthalimidinyl)-2-methoxyphenylsulfonyl chloride as a fluorescent labeling reagent. Anal Sci. 2008 Dec;24(12):1629-31. [PubMed ]

Brain injury (6 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB04659	8-Isoprostane	Blood; CSF; Urine	Bayir H, Kagan VE, Tyurina YY, Tyurin V, Ruppel RA, Adelson PD, Graham SH, Janesko K, Clark RS, Kochanek PM: Assessment of antioxidant reserves and oxidative stress in cerebrospinal fluid after severe traumatic brain injury in infants and children. Pediatr Res. 2002 May;51(5):571-8. [PubMed ]
HMDB00044	Ascorbic acid	Amniotic Fluid; Blood; Cellular Cytoplasm; CSF; Urine	Bayir H, Kagan VE, Tyurina YY, Tyurin V, Ruppel RA, Adelson PD, Graham SH, Janesko K, Clark RS, Kochanek PM: Assessment of antioxidant reserves and oxidative stress in cerebrospinal fluid after severe traumatic brain injury in infants and children. Pediatr Res. 2002 May;51(5):571-8. [PubMed ]
HMDB00125	Glutathione	Blood; Cellular Cytoplasm; CSF; Urine	Bayir H, Kagan VE, Tyurina YY, Tyurin V, Ruppel RA, Adelson PD, Graham SH, Janesko K, Clark RS, Kochanek PM: Assessment of antioxidant reserves and oxidative stress in cerebrospinal fluid after severe traumatic brain injury in infants and children. Pediatr Res. 2002 May;51(5):571-8. [PubMed ]
HMDB00224	O-Phosphoethanolamine	Blood; CSF; Urine	Seki Y, Kimura M, Mizutani N, Fujita M, Aimi Y, Suzuki Y: Cerebrospinal fluid taurine after traumatic brain injury. Neurochem Res. 2005 Jan;30(1):123-8. [PubMed ]
HMDB00070	Pipecolic acid	Amniotic Fluid; Blood; CSF; Urine	Smythe GA, Poljak A, Bustamante S, Braga O, Maxwell A, Grant R, Sachdev P: ECNI GC-MS analysis of picolinic and quinolinic acids and their amides in human plasma, CSF, and brain tissue. Adv Exp Med Biol. 2003;527:705-12. [PubMed ]
HMDB00232	Quinolinic acid	Blood; CSF; Urine	Smythe GA, Poljak A, Bustamante S, Braga O, Maxwell A, Grant R, Sachdev P: ECNI GC-MS analysis of picolinic and quinolinic acids and their amides in human plasma, CSF, and brain tissue. Adv Exp Med Biol. 2003;527:705-12. [PubMed ]

Brain tumors (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00582	3,5-Diiodothyronine	Blood; Urine	Pinna G, Meinhold H, Hiedra L, Thoma R, Hoell T, Graf KJ, Stoltenburg-Didinger G, Eravci M, Prengel H, Brodel O, Finke R, Baumgartner A: Elevated 3,5-diiodothyronine concentrations in the sera of patients with nonthyroidal illnesses and brain tumors. J Clin Endocrinol Metab. 1997 May;82(5):1535-42. [PubMed ]

Breast cancer (9 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00002	1,3-Diaminopropane	Blood; Urine	Byun JA, Lee SH, Jung BH, Choi MH, Moon MH, Chung BC: Analysis of polyamines as carbamoyl derivatives in urine and serum by liquid chromatography-tandem mass spectrometry. Biomed Chromatogr. 2008 Jan;22(1):73-80. [PubMed ]
HMDB01563	1-Methylguanosine	Blood; Urine	Zheng YF, Kong HW, Xiong JH, Lv S, Xu GW: Clinical significance and prognostic value of urinary nucleosides in breast cancer patients. Clin Biochem. 2005 Jan;38(1):24-30. [PubMed ]
HMDB00335	16a-Hydroxyestrone	Blood	Cauley JA, Zmuda JM, Danielson ME, Ljung BM, Bauer DC, Cummings SR, Kuller LH: Estrogen metabolites and the risk of breast cancer in older women. Epidemiology. 2003 Nov;14(6):740-4. [PubMed ]
HMDB05056	Enterodiol	Urine	Grace PB, Mistry NS, Carter MH, Leathem AJ, Teale P: High throughput quantification of phytoestrogens in human urine and serum using liquid chromatography/tandem mass spectrometry (LC-MS/MS). J Chromatogr B Analyt Technol Biomed Life Sci. 2007 Jun 15;853(1-2):138-46. Epub 2007 Mar 18. [PubMed ]
HMDB06101	Enterolactone	Urine	Grace PB, Mistry NS, Carter MH, Leathem AJ, Teale P: High throughput quantification of phytoestrogens in human urine and serum using liquid chromatography/tandem mass spectrometry (LC-MS/MS). J Chromatogr B Analyt Technol Biomed Life Sci. 2007 Jun 15;853(1-2):138-46. Epub 2007 Mar 18. [PubMed ]
HMDB01276	N1-Acetylspermidine	Blood; Urine	Byun JA, Lee SH, Jung BH, Choi MH, Moon MH, Chung BC: Analysis of polyamines as carbamoyl derivatives in urine and serum by liquid chromatography-tandem mass spectrometry. Biomed Chromatogr. 2008 Jan;22(1):73-80. [PubMed ]
HMDB02189	N8-Acetylspermidine	Blood; Urine	Byun JA, Lee SH, Jung BH, Choi MH, Moon MH, Chung BC: Analysis of polyamines as carbamoyl derivatives in urine and serum by liquid chromatography-tandem mass spectrometry. Biomed Chromatogr. 2008 Jan;22(1):73-80. [PubMed ]
HMDB04586	Perillic acid	Blood	Zhang Z, Chen H, Chan KK, Budd T, Ganapathi R: Gas chromatographic-mass spectrometric analysis of perillyl alcohol and metabolites in plasma. J Chromatogr B Biomed Sci Appl. 1999 May 14;728(1):85-95. [PubMed ]
HMDB00884	Ribothymidine	Blood; Urine	Zheng YF, Kong HW, Xiong JH, Lv S, Xu GW: Clinical significance and prognostic value of urinary nucleosides in breast cancer patients. Clin Biochem. 2005 Jan;38(1):24-30. [PubMed ]

Breast tumour (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00299	Xanthosine	Blood; Urine	Liebich HM, Di Stefano C, Wixforth A, Schmid HR: Quantitation of urinary nucleosides by high-performance liquid chromatography. J Chromatogr A. 1997 Feb 28;763(1-2):193-7. [PubMed ]

Cachexia (3 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00929	L-Tryptophan	Blood; CSF; Saliva; Urine
HMDB00158	L-Tyrosine	Blood; CSF; Saliva; Urine	Cachexia studies
HMDB00211	Myoinositol	Blood; CSF; Urine

Cadmium exposure (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00234	Testosterone	Blood; CSF; Saliva; Urine	Zeng X, Lin T, Zhou Y, Kong Q: Alterations of serum hormone levels in male workers occupationally exposed to cadmium. J Toxicol Environ Health A. 2002 Apr 12;65(7):513-21. [PubMed ]

Canavan disease (20 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB03099	1-Methyluric acid	Amniotic Fluid; Urine	Tavazzi B, Lazzarino G, Leone P, Amorini AM, Bellia F, Janson CG, Di Pietro V, Ceccarelli L, Donzelli S, Francis JS, Giardina B: Simultaneous high performance liquid chromatographic separation of purines, pyrimidines, N-acetylated amino acids, and dicarboxylic acids for the chemical diagnosis of inborn errors of metabolism. Clin Biochem. 2005 Nov;38(11):997-1008. Epub 2005 Sep 1. [PubMed ]
HMDB11600	3-Methyladenine	Urine	Tavazzi B, Lazzarino G, Leone P, Amorini AM, Bellia F, Janson CG, Di Pietro V, Ceccarelli L, Donzelli S, Francis JS, Giardina B: Simultaneous high performance liquid chromatographic separation of purines, pyrimidines, N-acetylated amino acids, and dicarboxylic acids for the chemical diagnosis of inborn errors of metabolism. Clin Biochem. 2005 Nov;38(11):997-1008. Epub 2005 Sep 1. [PubMed ]
HMDB00044	Ascorbic acid	Amniotic Fluid; Blood; Cellular Cytoplasm; CSF; Urine	Tavazzi B, Lazzarino G, Leone P, Amorini AM, Bellia F, Janson CG, Di Pietro V, Ceccarelli L, Donzelli S, Francis JS, Giardina B: Simultaneous high performance liquid chromatographic separation of purines, pyrimidines, N-acetylated amino acids, and dicarboxylic acids for the chemical diagnosis of inborn errors of metabolism. Clin Biochem. 2005 Nov;38(11):997-1008. Epub 2005 Sep 1. [PubMed ]
HMDB00094	Citric acid	Blood; CSF; Urine	Wevers RA, Engelke U, Wendel U, de Jong JG, Gabreels FJ, Heerschap A: Standardized method for high-resolution 1H-NMR of cerebrospinal fluid. Clin Chem. 1995 May;41(5):744-51. [PubMed ]
HMDB00562	Creatinine	Amniotic Fluid; Blood; CSF; Saliva; Urine	Tavazzi B, Lazzarino G, Leone P, Amorini AM, Bellia F, Janson CG, Di Pietro V, Ceccarelli L, Donzelli S, Francis JS, Giardina B: Simultaneous high performance liquid chromatographic separation of purines, pyrimidines, N-acetylated amino acids, and dicarboxylic acids for the chemical diagnosis of inborn errors of metabolism. Clin Biochem. 2005 Nov;38(11):997-1008. Epub 2005 Sep 1. [PubMed ]
HMDB00089	Cytidine	Blood; CSF; Urine	Tavazzi B, Lazzarino G, Leone P, Amorini AM, Bellia F, Janson CG, Di Pietro V, Ceccarelli L, Donzelli S, Francis JS, Giardina B: Simultaneous high performance liquid chromatographic separation of purines, pyrimidines, N-acetylated amino acids, and dicarboxylic acids for the chemical diagnosis of inborn errors of metabolism. Clin Biochem. 2005 Nov;38(11):997-1008. Epub 2005 Sep 1. [PubMed ]
HMDB00125	Glutathione	Blood; Cellular Cytoplasm; CSF; Urine	Tavazzi B, Lazzarino G, Leone P, Amorini AM, Bellia F, Janson CG, Di Pietro V, Ceccarelli L, Donzelli S, Francis JS, Giardina B: Simultaneous high performance liquid chromatographic separation of purines, pyrimidines, N-acetylated amino acids, and dicarboxylic acids for the chemical diagnosis of inborn errors of metabolism. Clin Biochem. 2005 Nov;38(11):997-1008. Epub 2005 Sep 1. [PubMed ]
HMDB00157	Hypoxanthine	Amniotic Fluid; Blood; Cellular Cytoplasm; CSF; Urine	Tavazzi B, Lazzarino G, Leone P, Amorini AM, Bellia F, Janson CG, Di Pietro V, Ceccarelli L, Donzelli S, Francis JS, Giardina B: Simultaneous high performance liquid chromatographic separation of purines, pyrimidines, N-acetylated amino acids, and dicarboxylic acids for the chemical diagnosis of inborn errors of metabolism. Clin Biochem. 2005 Nov;38(11):997-1008. Epub 2005 Sep 1. [PubMed ]
HMDB00195	Inosine	Blood; Cellular Cytoplasm; CSF; Pericardial Effusion; Urine	Tavazzi B, Lazzarino G, Leone P, Amorini AM, Bellia F, Janson CG, Di Pietro V, Ceccarelli L, Donzelli S, Francis JS, Giardina B: Simultaneous high performance liquid chromatographic separation of purines, pyrimidines, N-acetylated amino acids, and dicarboxylic acids for the chemical diagnosis of inborn errors of metabolism. Clin Biochem. 2005 Nov;38(11):997-1008. Epub 2005 Sep 1. [PubMed ]
HMDB00812	N-Acetyl-L-aspartic acid	Blood; CSF; Urine	Tavazzi B, Lazzarino G, Leone P, Amorini AM, Bellia F, Janson CG, Di Pietro V, Ceccarelli L, Donzelli S, Francis JS, Giardina B: Simultaneous high performance liquid chromatographic separation of purines, pyrimidines, N-acetylated amino acids, and dicarboxylic acids for the chemical diagnosis of inborn errors of metabolism. Clin Biochem. 2005 Nov;38(11):997-1008. Epub 2005 Sep 1. [PubMed ]
HMDB01067	N-Acetylaspartylglutamic acid	Blood; CSF; Urine	Burlina AP, Ferrari V, Divry P, Gradowska W, Jakobs C, Bennett MJ, Sewell AC, Dionisi-Vici C, Burlina AB: N-acetylaspartylglutamate in Canavan disease: an adverse effector? Eur J Pediatr. 1999 May;158(5):406-9. [PubMed ]
HMDB00226	Orotic acid	Amniotic Fluid; Blood; Urine	Tavazzi B, Lazzarino G, Leone P, Amorini AM, Bellia F, Janson CG, Di Pietro V, Ceccarelli L, Donzelli S, Francis JS, Giardina B: Simultaneous high performance liquid chromatographic separation of purines, pyrimidines, N-acetylated amino acids, and dicarboxylic acids for the chemical diagnosis of inborn errors of metabolism. Clin Biochem. 2005 Nov;38(11):997-1008. Epub 2005 Sep 1. [PubMed ]
HMDB03337	Oxidized glutathione	Blood; CSF	Tavazzi B, Lazzarino G, Leone P, Amorini AM, Bellia F, Janson CG, Di Pietro V, Ceccarelli L, Donzelli S, Francis JS, Giardina B: Simultaneous high performance liquid chromatographic separation of purines, pyrimidines, N-acetylated amino acids, and dicarboxylic acids for the chemical diagnosis of inborn errors of metabolism. Clin Biochem. 2005 Nov;38(11):997-1008. Epub 2005 Sep 1. [PubMed ]
HMDB00767	Pseudouridine	Amniotic Fluid; Blood; Urine	Tavazzi B, Lazzarino G, Leone P, Amorini AM, Bellia F, Janson CG, Di Pietro V, Ceccarelli L, Donzelli S, Francis JS, Giardina B: Simultaneous high performance liquid chromatographic separation of purines, pyrimidines, N-acetylated amino acids, and dicarboxylic acids for the chemical diagnosis of inborn errors of metabolism. Clin Biochem. 2005 Nov;38(11):997-1008. Epub 2005 Sep 1. [PubMed ]
HMDB00254	Succinic acid	Blood; CSF; Saliva; Urine	Wevers RA, Engelke U, Wendel U, de Jong JG, Gabreels FJ, Heerschap A: Standardized method for high-resolution 1H-NMR of cerebrospinal fluid. Clin Chem. 1995 May;41(5):744-51. [PubMed ]
HMDB00273	Thymidine	Amniotic Fluid; Blood; CSF; Urine	Tavazzi B, Lazzarino G, Leone P, Amorini AM, Bellia F, Janson CG, Di Pietro V, Ceccarelli L, Donzelli S, Francis JS, Giardina B: Simultaneous high performance liquid chromatographic separation of purines, pyrimidines, N-acetylated amino acids, and dicarboxylic acids for the chemical diagnosis of inborn errors of metabolism. Clin Biochem. 2005 Nov;38(11):997-1008. Epub 2005 Sep 1. [PubMed ]
HMDB00300	Uracil	Amniotic Fluid; Blood; CSF; Urine	Tavazzi B, Lazzarino G, Leone P, Amorini AM, Bellia F, Janson CG, Di Pietro V, Ceccarelli L, Donzelli S, Francis JS, Giardina B: Simultaneous high performance liquid chromatographic separation of purines, pyrimidines, N-acetylated amino acids, and dicarboxylic acids for the chemical diagnosis of inborn errors of metabolism. Clin Biochem. 2005 Nov;38(11):997-1008. Epub 2005 Sep 1. [PubMed ]
HMDB00289	Uric acid	Amniotic Fluid; Bile; Blood; CSF; Saliva; Urine	Tavazzi B, Lazzarino G, Leone P, Amorini AM, Bellia F, Janson CG, Di Pietro V, Ceccarelli L, Donzelli S, Francis JS, Giardina B: Simultaneous high performance liquid chromatographic separation of purines, pyrimidines, N-acetylated amino acids, and dicarboxylic acids for the chemical diagnosis of inborn errors of metabolism. Clin Biochem. 2005 Nov;38(11):997-1008. Epub 2005 Sep 1. [PubMed ]
HMDB00296	Uridine	Blood; CSF; Urine	Tavazzi B, Lazzarino G, Leone P, Amorini AM, Bellia F, Janson CG, Di Pietro V, Ceccarelli L, Donzelli S, Francis JS, Giardina B: Simultaneous high performance liquid chromatographic separation of purines, pyrimidines, N-acetylated amino acids, and dicarboxylic acids for the chemical diagnosis of inborn errors of metabolism. Clin Biochem. 2005 Nov;38(11):997-1008. Epub 2005 Sep 1. [PubMed ]
HMDB00292	Xanthine	Blood; CSF; Urine	Tavazzi B, Lazzarino G, Leone P, Amorini AM, Bellia F, Janson CG, Di Pietro V, Ceccarelli L, Donzelli S, Francis JS, Giardina B: Simultaneous high performance liquid chromatographic separation of purines, pyrimidines, N-acetylated amino acids, and dicarboxylic acids for the chemical diagnosis of inborn errors of metabolism. Clin Biochem. 2005 Nov;38(11):997-1008. Epub 2005 Sep 1. [PubMed ]

Cancer (4 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB02721	1-Methylinosine	Blood; Urine	Liebich HM, Di Stefano C, Wixforth A, Schmid HR: Quantitation of urinary nucleosides by high-performance liquid chromatography. J Chromatogr A. 1997 Feb 28;763(1-2):193-7. [PubMed ]
HMDB01388	Alpha-Linolenic acid	Blood; Urine	Kim KM, Jung BH, Lho DS, Chung WY, Paeng KJ, Chung BC: Alteration of urinary profiles of endogenous steroids and polyunsaturated fatty acids in thyroid cancer. Cancer Lett. 2003 Dec 30;202(2):173-9. [PubMed ]
HMDB01893	Alpha-Tocopherol	Blood; Breast Milk; CSF; Urine	Jeong NH, Song ES, Lee JM, Lee KB, Kim MK, Yun YM, Lee JK, Son SK, Lee JP, Kim JH, Hur SY, Kwon YI: Preoperative levels of plasma micronutrients are related to endometrial cancer risk. Acta Obstet Gynecol Scand. 2009;88(4):434-9. [PubMed ]
HMDB00305	Vitamin A	Blood	Jeong NH, Song ES, Lee JM, Lee KB, Kim MK, Yun YM, Lee JK, Son SK, Lee JP, Kim JH, Hur SY, Kwon YI: Preoperative levels of plasma micronutrients are related to endometrial cancer risk. Acta Obstet Gynecol Scand. 2009;88(4):434-9. [PubMed ]

Cancer - assorted types (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00174	L-Fucose	Blood; Breast Milk; Urine	Sakai T, Yamamoto K, Yokota H, Hakozaki-Usui K, Hino F, Kato I: Rapid, simple enzymatic assay of free L-fucose in serum and urine, and its use as a marker for cancer, cirrhosis, and gastric ulcers. Clin Chem. 1990 Mar;36(3):474-6. [PubMed ]

Carbamoyl phosphate synthetase deficiency (4 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00123	Glycine	Bile; Blood; CSF; Saliva; Urine	Metagene: CARBAMOYL PHOSPHATE SYNTHETASE DEFICIENCY (CPS)
HMDB00641	L-Glutamine	Blood; CSF; Saliva; Urine	Metagene: CARBAMOYL PHOSPHATE SYNTHETASE DEFICIENCY (CPS)
HMDB00182	L-Lysine	Blood; CSF; Saliva; Urine	Metagene: CARBAMOYL PHOSPHATE SYNTHETASE DEFICIENCY (CPS)
HMDB00300	Uracil	Amniotic Fluid; Blood; CSF; Urine	Metagene: CARBAMOYL PHOSPHATE SYNTHETASE DEFICIENCY (CPS)

Carcinoid tumors (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00763	5-Hydroxyindoleacetic acid	Blood; CSF; Urine	Ishida J, Iizuka R, Yamaguchi M: High-performance liquid chromatographic determination of 5-hydroxyindoles by post-column fluorescence derivatization. Analyst. 1993 Feb;118(2):165-9. [PubMed ]

Cardiac arrest (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB01139	Prostaglandin F2a	Blood; CSF; Urine	Strohmenger HU, Lindner KH, Keller A, Lindner IM, Bothner U, Georgieff M: Concentrations of prolactin and prostaglandins during and after cardiopulmonary resuscitation. Crit Care Med. 1995 Aug;23(8):1347-55. [PubMed ]

Cardiopulmonary bypass (2 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB01085	Leukotriene B4	Blood; CSF; Urine	Pearl JM, Manning PB, McNamara JL, Saucier MM, Thomas DW: Effect of modified ultrafiltration on plasma thromboxane B2, leukotriene B4, and endothelin-1 in infants undergoing cardiopulmonary bypass. Ann Thorac Surg. 1999 Oct;68(4):1369-75. [PubMed ]
HMDB03378	Nitric oxide	Blood; CSF; Urine	Duke T, South M, Stewart A: Altered activation of the L-arginine nitric oxide pathway during and after cardiopulmonary bypass. Perfusion. 1997 Nov;12(6):405-10. [PubMed ]

Cardiopulmonary resuscitation (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB02886	6-Keto-prostaglandin F1a	Blood; Urine	Strohmenger HU, Lindner KH, Keller A, Lindner IM, Bothner U, Georgieff M: Concentrations of prolactin and prostaglandins during and after cardiopulmonary resuscitation. Crit Care Med. 1995 Aug;23(8):1347-55. [PubMed ]

Carnitine-Acylcarnitine translocase deficiency (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00792	Sebacic acid	Urine	Metagene: CARNITINE-ACYLCARNITINE TRANSLOCASE DEFICIENCY

Carnosinuria (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00033	Carnosine	Blood; Urine	Metagene: CARNOSINURIA

Celiac Disease (12 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB02013	Butyrylcarnitine	Blood	Bene J, Komlosi K, Gasztonyi B, Juhasz M, Tulassay Z, Melegh B: Plasma carnitine ester profile in adult celiac disease patients maintained on long-term gluten free diet. World J Gastroenterol. 2005 Nov 14;11(42):6671-5. [PubMed ]
HMDB00651	Decanoylcarnitine	Blood	Bene J, Komlosi K, Gasztonyi B, Juhasz M, Tulassay Z, Melegh B: Plasma carnitine ester profile in adult celiac disease patients maintained on long-term gluten free diet. World J Gastroenterol. 2005 Nov 14;11(42):6671-5. [PubMed ]
HMDB02250	Dodecanoylcarnitine	Blood	Bene J, Komlosi K, Gasztonyi B, Juhasz M, Tulassay Z, Melegh B: Plasma carnitine ester profile in adult celiac disease patients maintained on long-term gluten free diet. World J Gastroenterol. 2005 Nov 14;11(42):6671-5. [PubMed ]
HMDB00138	Glycocholic acid	Blood; Urine	Spiller RC, Frost PF, Stewart JS, Bloom SR, Silk DB: Delayed postprandial plasma bile acid response in coeliac patients with slow mouth-caecum transit. Clin Sci (Lond). 1987 Feb;72(2):217-23. [PubMed ]
HMDB00688	Isovalerylcarnitine	Blood	Bene J, Komlosi K, Gasztonyi B, Juhasz M, Tulassay Z, Melegh B: Plasma carnitine ester profile in adult celiac disease patients maintained on long-term gluten free diet. World J Gastroenterol. 2005 Nov 14;11(42):6671-5. [PubMed ]
HMDB00756	L-Hexanoylcarnitine	Blood	Bene J, Komlosi K, Gasztonyi B, Juhasz M, Tulassay Z, Melegh B: Plasma carnitine ester profile in adult celiac disease patients maintained on long-term gluten free diet. World J Gastroenterol. 2005 Nov 14;11(42):6671-5. [PubMed ]
HMDB00791	L-Octanoylcarnitine	Blood	Bene J, Komlosi K, Gasztonyi B, Juhasz M, Tulassay Z, Melegh B: Plasma carnitine ester profile in adult celiac disease patients maintained on long-term gluten free diet. World J Gastroenterol. 2005 Nov 14;11(42):6671-5. [PubMed ]
HMDB00222	L-Palmitoylcarnitine	Blood; Urine	Bene J, Komlosi K, Gasztonyi B, Juhasz M, Tulassay Z, Melegh B: Plasma carnitine ester profile in adult celiac disease patients maintained on long-term gluten free diet. World J Gastroenterol. 2005 Nov 14;11(42):6671-5. [PubMed ]
HMDB00740	Lactulose	Blood	Cox MA, Lewis KO, Cooper BT: Measurement of small intestinal permeability markers, lactulose, and mannitol in serum: results in celiac disease. Dig Dis Sci. 1999 Feb;44(2):402-6. [PubMed ]
HMDB00824	Propionylcarnitine	Blood	Bene J, Komlosi K, Gasztonyi B, Juhasz M, Tulassay Z, Melegh B: Plasma carnitine ester profile in adult celiac disease patients maintained on long-term gluten free diet. World J Gastroenterol. 2005 Nov 14;11(42):6671-5. [PubMed ]
HMDB00848	Stearoylcarnitine	Blood	Bene J, Komlosi K, Gasztonyi B, Juhasz M, Tulassay Z, Melegh B: Plasma carnitine ester profile in adult celiac disease patients maintained on long-term gluten free diet. World J Gastroenterol. 2005 Nov 14;11(42):6671-5. [PubMed ]
HMDB02366	Tiglylcarnitine	Blood	Bene J, Komlosi K, Gasztonyi B, Juhasz M, Tulassay Z, Melegh B: Plasma carnitine ester profile in adult celiac disease patients maintained on long-term gluten free diet. World J Gastroenterol. 2005 Nov 14;11(42):6671-5. [PubMed ]

Cerebral infarction (2 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00076	Dihydrouracil	Blood; CSF; Urine	Hayashi K, Kidouchi K, Sumi S, Mizokami M, Orito E, Kumada K, Ueda R, Wada Y: Possible prediction of adverse reactions to pyrimidine chemotherapy from urinary pyrimidine levels and a case of asymptomatic adult dihydropyrimidinuria. Clin Cancer Res. 1996 Dec;2(12):1937-41. [PubMed ]
HMDB00300	Uracil	Amniotic Fluid; Blood; CSF; Urine	Hayashi K, Kidouchi K, Sumi S, Mizokami M, Orito E, Kumada K, Ueda R, Wada Y: Possible prediction of adverse reactions to pyrimidine chemotherapy from urinary pyrimidine levels and a case of asymptomatic adult dihydropyrimidinuria. Clin Cancer Res. 1996 Dec;2(12):1937-41. [PubMed ]

Cerebral Vasospasm (3 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB06111	12-HETE	Blood; CSF; Urine	Poloyac SM, Reynolds RB, Yonas H, Kerr ME: Identification and quantification of the hydroxyeicosatetraenoic acids, 20-HETE and 12-HETE, in the cerebrospinal fluid after subarachnoid hemorrhage. J Neurosci Methods. 2005 Jun 15;144(2):257-63. Epub 2004 Dec 30. [PubMed ]
HMDB05998	20-Hydroxyeicosatetraenoic acid	Blood; CSF; Urine	Poloyac SM, Reynolds RB, Yonas H, Kerr ME: Identification and quantification of the hydroxyeicosatetraenoic acids, 20-HETE and 12-HETE, in the cerebrospinal fluid after subarachnoid hemorrhage. J Neurosci Methods. 2005 Jun 15;144(2):257-63. Epub 2004 Dec 30. [PubMed ]
HMDB00054	Bilirubin	Bile; Blood; CSF; Urine	Pyne-Geithman GJ, Morgan CJ, Wagner K, Dulaney EM, Carrozzella J, Kanter DS, Zuccarello M, Clark JF: Bilirubin production and oxidation in CSF of patients with cerebral vasospasm after subarachnoid hemorrhage. J Cereb Blood Flow Metab. 2005 Aug;25(8):1070-7. [PubMed ]

Cerebrocortical Degeneration (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB01372	Thiamine pyrophosphate	Blood; CSF	Holtershinken M, Hohling A, Witte B, Scholz H: [Thiamine and its derivates in cattle blood measured by HPLC in healthy animals, in patients suffering from CCN and in their cohorts] Dtsch Tierarztl Wochenschr. 2007 Jun;114(6):212-8. [PubMed ]

Cerebrotendinous xanthomatosis (CTX) (2 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00908	5alpha-Cholestanol	Blood	Halperin G, Elisaf M, Leitersdorf E, Harats D: A new method for determination of serum cholestanol by high-performance liquid chromatography with ultraviolet detection. J Chromatogr B Biomed Sci Appl. 2000 Jun 9;742(2):345-52. [PubMed ]
HMDB01170	Lathosterol	Blood	Halperin G, Elisaf M, Leitersdorf E, Harats D: A new method for determination of serum cholestanol by high-performance liquid chromatography with ultraviolet detection. J Chromatogr B Biomed Sci Appl. 2000 Jun 9;742(2):345-52. [PubMed ]

Cervical cancer (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB03331	1-Methyladenosine	Blood; Urine	Ishiwata S, Itoh K, Yamaguchi T, Ishida N, Mizugaki M: Comparison of serum and urinary levels of modified nucleoside, 1-methyladenosine, in cancer patients using a monoclonal antibody-based inhibition ELISA. Tohoku J Exp Med. 1995 May;176(1):61-8. [PubMed ]

Cervical myelopathy (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00108	Ethanol	Blood; CSF; Saliva; Urine	Meshitsuka S, Morio Y, Nagashima H, Teshima R: 1H-NMR studies of cerebrospinal fluid: endogenous ethanol in patients with cervical myelopathy. Clin Chim Acta. 2001 Oct;312(1-2):25-30. [PubMed ]

Cholangioma cancer (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB03331	1-Methyladenosine	Blood; Urine	Ishiwata S, Itoh K, Yamaguchi T, Ishida N, Mizugaki M: Comparison of serum and urinary levels of modified nucleoside, 1-methyladenosine, in cancer patients using a monoclonal antibody-based inhibition ELISA. Tohoku J Exp Med. 1995 May;176(1):61-8. [PubMed ]

Cholecohal cyst (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00138	Glycocholic acid	Blood; Urine	Matsui A, Psacharopoulos HT, Mowat AP, Portmann B, Murphy GM: Radioimmunoassay of serum glycocholic acid, standard laboratory tests of liver function and liver biopsy findings: comparative study of children with liver disease. J Clin Pathol. 1982 Sep;35(9):1011-7. [PubMed ]

Cholesterol stones (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00067	Cholesterol	Bile; Blood; CSF; Saliva; Urine	Miettinen TE, Kesaniemi YA, Gylling H, Jarvinen H, Silvennoinen E, Miettinen TA: Noncholesterol sterols in bile and stones of patients with cholesterol and pigment stones. Hepatology. 1996 Feb;23(2):274-80. [PubMed ]

Cholesteryl ester storage disease (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00067	Cholesterol	Bile; Blood; CSF; Saliva; Urine	Metagene: CHOLESTERYL ESTER STORAGE DISEASE

Chronic active hepatitis (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00138	Glycocholic acid	Blood; Urine	Matsui A, Psacharopoulos HT, Mowat AP, Portmann B, Murphy GM: Radioimmunoassay of serum glycocholic acid, standard laboratory tests of liver function and liver biopsy findings: comparative study of children with liver disease. J Clin Pathol. 1982 Sep;35(9):1011-7. [PubMed ]

Chronic progressive external opthalmoplegia (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00190	L-Lactic acid	Bile; Blood; Cellular Cytoplasm; CSF; Urine	Metagene: CHRONIC PROGRESSIVE EXTERNAL OPHTHALMOPLEGIA AND KEARNS-SAYRE SYNDROM

Chronic renal disease (2 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB03474	3,5-Diiodo-L-tyrosine	Blood; Urine	Ramsden DB, Farmer M, Mohammed MN, McGonigle RS, Sheppard MC: 3,5-Diiodotyrosine and thyronine in the urine of patients with chronic renal disease. Clin Endocrinol (Oxf). 1986 May;24(5):491-5. [PubMed ]
HMDB00667	L-Thyronine	Blood; Urine	Ramsden DB, Farmer M, Mohammed MN, McGonigle RS, Sheppard MC: 3,5-Diiodotyrosine and thyronine in the urine of patients with chronic renal disease. Clin Endocrinol (Oxf). 1986 May;24(5):491-5. [PubMed ]

Chronic renal failure (11 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00043	Betaine	Blood; Urine	McGregor DO, Dellow WJ, Lever M, George PM, Robson RA, Chambers ST: Dimethylglycine accumulates in uremia and predicts elevated plasma homocysteine concentrations. Kidney Int. 2001 Jun;59(6):2267-72. [PubMed ]
HMDB00562	Creatinine	Amniotic Fluid; Blood; CSF; Saliva; Urine	Kikuchi T, Orita Y, Ando A, Mikami H, Fujii M, Okada A, Abe H: Liquid-chromatographic determination of guanidino compounds in plasma and erythrocyte of normal persons and uremic patients. Clin Chem. 1981 Nov;27(11):1899-902. [PubMed ]
HMDB00058	Cyclic AMP	Blood; CSF; Urine	Dimitrakov D, Kumchev E, Lyutakova E, Ledzhev I, Nikolov D, Tsekov V: Cyclic adenosine monophosphate (cAMP) levels in predialysis patients with chronic renal failure. Folia Med (Plovdiv). 1997;39(1):29-33. [PubMed ]
HMDB01842	Guanidine	Blood; CSF; Urine	Kikuchi T, Orita Y, Ando A, Mikami H, Fujii M, Okada A, Abe H: Liquid-chromatographic determination of guanidino compounds in plasma and erythrocyte of normal persons and uremic patients. Clin Chem. 1981 Nov;27(11):1899-902. [PubMed ]
HMDB03157	Guanidinosuccinic acid	Blood; Cellular Cytoplasm; CSF; Urine	Kikuchi T, Orita Y, Ando A, Mikami H, Fujii M, Okada A, Abe H: Liquid-chromatographic determination of guanidino compounds in plasma and erythrocyte of normal persons and uremic patients. Clin Chem. 1981 Nov;27(11):1899-902. [PubMed ]
HMDB00128	Guanidoacetic acid	Blood; Cellular Cytoplasm; CSF; Urine	Kikuchi T, Orita Y, Ando A, Mikami H, Fujii M, Okada A, Abe H: Liquid-chromatographic determination of guanidino compounds in plasma and erythrocyte of normal persons and uremic patients. Clin Chem. 1981 Nov;27(11):1899-902. [PubMed ]
HMDB00742	Homocysteine	Blood; CSF; Urine	McGregor DO, Dellow WJ, Lever M, George PM, Robson RA, Chambers ST: Dimethylglycine accumulates in uremia and predicts elevated plasma homocysteine concentrations. Kidney Int. 2001 Jun;59(6):2267-72. [PubMed ]
HMDB00517	L-Arginine	Blood; CSF; Urine	Kikuchi T, Orita Y, Ando A, Mikami H, Fujii M, Okada A, Abe H: Liquid-chromatographic determination of guanidino compounds in plasma and erythrocyte of normal persons and uremic patients. Clin Chem. 1981 Nov;27(11):1899-902. [PubMed ]
HMDB04068	Melanin	Blood	Hegedus ZL, Frank HA, Steinman TI, Altschule MD, Nayak U: Elevated levels of plasma lipofuscins in patients with chronic renal failure. Arch Int Physiol Biochim. 1988 Dec;96(5):211-21. [PubMed ]
HMDB01522	Methylguanidine	Blood; CSF; Saliva; Urine	Kikuchi T, Orita Y, Ando A, Mikami H, Fujii M, Okada A, Abe H: Liquid-chromatographic determination of guanidino compounds in plasma and erythrocyte of normal persons and uremic patients. Clin Chem. 1981 Nov;27(11):1899-902. [PubMed ]
HMDB01303	Zinc	Blood; CSF; Urine	McGregor DO, Dellow WJ, Lever M, George PM, Robson RA, Chambers ST: Dimethylglycine accumulates in uremia and predicts elevated plasma homocysteine concentrations. Kidney Int. 2001 Jun;59(6):2267-72. [PubMed ]

Circulatory system disorder (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB02243	Picolinic acid	Blood; CSF	Coggan SE, Smythe GA, Bilgin A, Grant RS: Age and circadian influences on picolinic acid concentrations in human cerebrospinal fluid. J Neurochem. 2009 Mar;108(5):1220-5. Epub 2009 Jan 28. [PubMed ]

Cirrhosis (23 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB03156	2,3-Butanediol	Blood	Casazza JP, Frietas J, Stambuk D, Morgan MY, Veech RL: The measurement of 1,2-propanediol, D, L-2,3-butanediol and meso-2,3-butanediol in controls and alcoholic cirrhotics. Alcohol Alcohol Suppl. 1987;1:607-9. [PubMed ]
HMDB01065	2-Hydroxyphenethylamine	Blood; Urine	Yonekura T, Kamata S, Wasa M, Okada A, Yamatodani A, Watanabe T, Wada H: Simultaneous determination of plasma phenethylamine, phenylethanolamine, tyramine and octopamine by high-performance liquid chromatography using derivatization with fluorescamine. J Chromatogr. 1988 Jun 3;427(2):320-5. [PubMed ]
HMDB04225	2-Oxoarginine	Blood; CSF	Marescau B, De Deyn PP, Holvoet J, Possemiers I, Nagels G, Saxena V, Mahler C: Guanidino compounds in serum and urine of cirrhotic patients. Metabolism. 1995 May;44(5):584-8. [PubMed ]
HMDB00582	3,5-Diiodothyronine	Blood; Urine	Pinna G, Meinhold H, Hiedra L, Thoma R, Hoell T, Graf KJ, Stoltenburg-Didinger G, Eravci M, Prengel H, Brodel O, Finke R, Baumgartner A: Elevated 3,5-diiodothyronine concentrations in the sera of patients with nonthyroidal illnesses and brain tumors. J Clin Endocrinol Metab. 1997 May;82(5):1535-42. [PubMed ]
HMDB03464	4-Guanidinobutanoic acid	Blood; CSF; Urine	Marescau B, De Deyn PP, Holvoet J, Possemiers I, Nagels G, Saxena V, Mahler C: Guanidino compounds in serum and urine of cirrhotic patients. Metabolism. 1995 May;44(5):584-8. [PubMed ]
HMDB00908	5alpha-Cholestanol	Blood	Halperin G, Elisaf M, Leitersdorf E, Harats D: A new method for determination of serum cholestanol by high-performance liquid chromatography with ultraviolet detection. J Chromatogr B Biomed Sci Appl. 2000 Jun 9;742(2):345-52. [PubMed ]
HMDB01496	7a-Hydroxycholesterol	Blood; CSF	Hahn C, Reichel C, von Bergmann K: Serum concentration of 7 alpha-hydroxycholesterol as an indicator of bile acid synthesis in humans. J Lipid Res. 1995 Sep;36(9):2059-66. [PubMed ]
HMDB03148	Argininic acid	Blood	Marescau B, De Deyn PP, Holvoet J, Possemiers I, Nagels G, Saxena V, Mahler C: Guanidino compounds in serum and urine of cirrhotic patients. Metabolism. 1995 May;44(5):584-8. [PubMed ]
HMDB00653	Cholesterol sulfate	Blood	Tamasawa N, Tamasawa A, Takebe K: Higher levels of plasma cholesterol sulfate in patients with liver cirrhosis and hypercholesterolemia. Lipids. 1993 Sep;28(9):833-6. [PubMed ]
HMDB00064	Creatine	Blood; CSF; Urine	Marescau B, De Deyn PP, Holvoet J, Possemiers I, Nagels G, Saxena V, Mahler C: Guanidino compounds in serum and urine of cirrhotic patients. Metabolism. 1995 May;44(5):584-8. [PubMed ]
HMDB01045	Enkephalin L	Blood	Thornton JR, Losowsky MS: Plasma leucine enkephalin is increased in liver disease. Gut. 1989 Oct;30(10):1392-5. [PubMed ]
HMDB01842	Guanidine	Blood; CSF; Urine	Marescau B, De Deyn PP, Holvoet J, Possemiers I, Nagels G, Saxena V, Mahler C: Guanidino compounds in serum and urine of cirrhotic patients. Metabolism. 1995 May;44(5):584-8. [PubMed ]
HMDB03157	Guanidinosuccinic acid	Blood; Cellular Cytoplasm; CSF; Urine	Marescau B, De Deyn PP, Holvoet J, Possemiers I, Nagels G, Saxena V, Mahler C: Guanidino compounds in serum and urine of cirrhotic patients. Metabolism. 1995 May;44(5):584-8. [PubMed ]
HMDB00670	Homo-L-arginine	Blood; CSF; Urine	Marescau B, De Deyn PP, Holvoet J, Possemiers I, Nagels G, Saxena V, Mahler C: Guanidino compounds in serum and urine of cirrhotic patients. Metabolism. 1995 May;44(5):584-8. [PubMed ]
HMDB00191	L-Aspartic acid	Blood; CSF; Saliva; Urine	Cynober LA: Plasma amino acid levels with a note on membrane transport: characteristics, regulation, and metabolic significance. Nutrition. 2002 Sep;18(9):761-6. [PubMed ]
HMDB01170	Lathosterol	Blood	Halperin G, Elisaf M, Leitersdorf E, Harats D: A new method for determination of serum cholestanol by high-performance liquid chromatography with ultraviolet detection. J Chromatogr B Biomed Sci Appl. 2000 Jun 9;742(2):345-52. [PubMed ]
HMDB00673	Linoleic acid	Blood; CSF; Urine	Szebeni J, Eskelson C, Sampliner R, Hartmann B, Griffin J, Dormandy T, Watson RR: Plasma fatty acid pattern including diene-conjugated linoleic acid in ethanol users and patients with ethanol-related liver disease. Alcohol Clin Exp Res. 1986 Dec;10(6):647-50. [PubMed ]
HMDB03227	Methanethiol	Blood	McClain CJ, Zieve L, Doizaki WM, Gilberstadt S, Onstad GR: Blood methanethiol in alcoholic liver disease with and without hepatic encephalopathy. Gut. 1980 Apr;21(4):318-23. [PubMed ]
HMDB04825	p-Octopamine	Blood; Urine	Yonekura T, Kamata S, Wasa M, Okada A, Yamatodani A, Watanabe T, Wada H: Simultaneous determination of plasma phenethylamine, phenylethanolamine, tyramine and octopamine by high-performance liquid chromatography using derivatization with fluorescamine. J Chromatogr. 1988 Jun 3;427(2):320-5. [PubMed ]
HMDB01881	Propylene glycol	Blood; CSF; Saliva; Urine	Casazza JP, Frietas J, Stambuk D, Morgan MY, Veech RL: The measurement of 1,2-propanediol, D, L-2,3-butanediol and meso-2,3-butanediol in controls and alcoholic cirrhotics. Alcohol Alcohol Suppl. 1987;1:607-9. [PubMed ]
HMDB02639	Sulfolithocholylglycine	Blood; Urine	Kobayashi N, Katsumata H, Uto Y, Goto J, Niwa T, Kobayashi K, Mizuuchi Y: A monoclonal antibody-based enzyme-linked immunosorbent assay of glycolithocholic acid sulfate in human urine for liver function test. Steroids. 2002 Sep;67(10):827-33. [PubMed ]
HMDB00306	Tyramine	Blood; Urine	Yonekura T, Kamata S, Wasa M, Okada A, Yamatodani A, Watanabe T, Wada H: Simultaneous determination of plasma phenethylamine, phenylethanolamine, tyramine and octopamine by high-performance liquid chromatography using derivatization with fluorescamine. J Chromatogr. 1988 Jun 3;427(2):320-5. [PubMed ]
HMDB00294	Urea	Blood; Cellular Cytoplasm; CSF; Urine	Marescau B, De Deyn PP, Holvoet J, Possemiers I, Nagels G, Saxena V, Mahler C: Guanidino compounds in serum and urine of cirrhotic patients. Metabolism. 1995 May;44(5):584-8. [PubMed ]

Cisplatin containing chemotherapy (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB01897	Substance P	Blood; Urine	Higa GM, Auber ML, Altaha R, Piktel D, Kurian S, Hobbs G, Landreth K: 5-Hydroxyindoleacetic acid and substance P profiles in patients receiving emetogenic chemotherapy. J Oncol Pharm Pract. 2006 Dec;12(4):201-9. [PubMed ]

Citrullinemia Type I (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00051	Ammonia	Blood; Cellular Cytoplasm; CSF; Urine	Metagene: CITRULLINEMIA TYPE I

CNS Infections (3 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00684	L-Kynurenine	Blood; CSF; Urine	Heyes MP, Saito K, Milstien S, Schiff SJ: Quinolinic acid in tumors, hemorrhage and bacterial infections of the central nervous system in children. J Neurol Sci. 1995 Nov;133(1-2):112-8. [PubMed ]
HMDB00845	Neopterin	Blood; CSF; Urine	Heyes MP, Saito K, Milstien S, Schiff SJ: Quinolinic acid in tumors, hemorrhage and bacterial infections of the central nervous system in children. J Neurol Sci. 1995 Nov;133(1-2):112-8. [PubMed ]
HMDB00232	Quinolinic acid	Blood; CSF; Urine	Heyes MP, Saito K, Milstien S, Schiff SJ: Quinolinic acid in tumors, hemorrhage and bacterial infections of the central nervous system in children. J Neurol Sci. 1995 Nov;133(1-2):112-8. [PubMed ]

CNS Tumors (3 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00684	L-Kynurenine	Blood; CSF; Urine	Heyes MP, Saito K, Milstien S, Schiff SJ: Quinolinic acid in tumors, hemorrhage and bacterial infections of the central nervous system in children. J Neurol Sci. 1995 Nov;133(1-2):112-8. [PubMed ]
HMDB00845	Neopterin	Blood; CSF; Urine	Heyes MP, Saito K, Milstien S, Schiff SJ: Quinolinic acid in tumors, hemorrhage and bacterial infections of the central nervous system in children. J Neurol Sci. 1995 Nov;133(1-2):112-8. [PubMed ]
HMDB00232	Quinolinic acid	Blood; CSF; Urine	Heyes MP, Saito K, Milstien S, Schiff SJ: Quinolinic acid in tumors, hemorrhage and bacterial infections of the central nervous system in children. J Neurol Sci. 1995 Nov;133(1-2):112-8. [PubMed ]

Cobalamin Deficiency (3 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB03640	Beta-Leucine	Blood	Stabler SP, Lindenbaum J, Allen RH: Failure to detect beta-leucine in human blood or leucine 2,3-aminomutase in rat liver using capillary gas chromatography-mass spectrometry. J Biol Chem. 1988 Apr 25;263(12):5581-8. [PubMed ]
HMDB00099	L-Cystathionine	Blood; CSF; Urine	Stabler SP, Lindenbaum J, Savage DG, Allen RH: Elevation of serum cystathionine levels in patients with cobalamin and folate deficiency. Blood. 1993 Jun 15;81(12):3404-13. [PubMed ]
HMDB00202	Methylmalonic acid	Blood; CSF; Urine	Hoffmann GF, Meier-Augenstein W, Stockler S, Surtees R, Rating D, Nyhan WL: Physiology and pathophysiology of organic acids in cerebrospinal fluid. J Inherit Metab Dis. 1993;16(4):648-69. [PubMed ]

Cobalamin malabsorption (2 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB03429	Cob(I)alamin	Blood	Metagene: COBALAMIN MALABSORPTION
HMDB00202	Methylmalonic acid	Blood; CSF; Urine	Metagene: COBALAMIN MALABSORPTION

Cognitive Disorders (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB01490	Vanylglycol	Blood; CSF; Urine	Hildebrand J, Bourgeois F, Buyse M, Przedborski S, Goldman S: Reproducibility of monoamine metabolite measurements in human cerebrospinal fluid. Acta Neurol Scand. 1990 May;81(5):427-30. [PubMed ]

Colitis (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB04866	Lactosylceramide (d18:1/12:0)	Blood	Stevens CR, Oberholzer VG, Walker-Smith JA, Phillips AD: Lactosylceramide in inflammatory bowel disease: a biochemical study. Gut. 1988 May;29(5):580-7. [PubMed ]

Colon cancer (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB03331	1-Methyladenosine	Blood; Urine	Ishiwata S, Itoh K, Yamaguchi T, Ishida N, Mizugaki M: Comparison of serum and urinary levels of modified nucleoside, 1-methyladenosine, in cancer patients using a monoclonal antibody-based inhibition ELISA. Tohoku J Exp Med. 1995 May;176(1):61-8. [PubMed ]

Complex febrile seizures (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00175	Inosinic acid	Blood; Cellular Cytoplasm; CSF	Rodriguez-Nunez A, Cid E, Rodriguez-Garcia J, Camina F, Rodriguez-Segade S, Castro-Gago M: Cerebrospinal fluid purine metabolite and neuron-specific enolase concentrations after febrile seizures. Brain Dev. 2000 Oct;22(7):427-31. [PubMed ]

Congenital adrenal hyperplasia (2 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00315	16-a-Hydroxypregnenolone	Blood; Urine	Homoki J, Teller WM: Increased urinary excretion of total 16 alpha-hydroxypregnenolone in newborn infants with congenital adrenal hyperplasia due to 21-hydroxylase deficiency. Klin Wochenschr. 1982 Apr 15;60(8):407-10. [PubMed ]
HMDB04030	21-Deoxycortisol	Blood	Janzen N, Peter M, Sander S, Steuerwald U, Terhardt M, Holtkamp U, Sander J: Newborn screening for congenital adrenal hyperplasia: additional steroid profile using liquid chromatography-tandem mass spectrometry. J Clin Endocrinol Metab. 2007 Jul;92(7):2581-9. Epub 2007 Apr 24. [PubMed ]

Congestive heart failure (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00851	Pyridinoline	Blood; Urine	Shane E, Mancini D, Aaronson K, Silverberg SJ, Seibel MJ, Addesso V, McMahon DJ: Bone mass, vitamin D deficiency, and hyperparathyroidism in congestive heart failure. Am J Med. 1997 Sep;103(3):197-207. [PubMed ]

Continuous ambulatory peritoneal dialysis (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00043	Betaine	Blood; Urine	McGregor DO, Dellow WJ, Lever M, George PM, Robson RA, Chambers ST: Dimethylglycine accumulates in uremia and predicts elevated plasma homocysteine concentrations. Kidney Int. 2001 Jun;59(6):2267-72. [PubMed ]

Continuous ambulatory peritoneal dialysis (2 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00870	Histamine	Blood; CSF; Urine	Gill DS, Fonseca VA, Barradas MA, Balliod R, Moorhead JF, Dandona P: Plasma histamine in patients with chronic renal failure and nephrotic syndrome. J Clin Pathol. 1991 Mar;44(3):243-5. [PubMed ]
HMDB01303	Zinc	Blood; CSF; Urine	McGregor DO, Dellow WJ, Lever M, George PM, Robson RA, Chambers ST: Dimethylglycine accumulates in uremia and predicts elevated plasma homocysteine concentrations. Kidney Int. 2001 Jun;59(6):2267-72. [PubMed ]

Continuous ambulatory peritoneal dialysis (CAPD) (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00742	Homocysteine	Blood; CSF; Urine	McGregor DO, Dellow WJ, Lever M, George PM, Robson RA, Chambers ST: Dimethylglycine accumulates in uremia and predicts elevated plasma homocysteine concentrations. Kidney Int. 2001 Jun;59(6):2267-72. [PubMed ]

Convulsions (3 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00715	Kynurenic acid	Blood; CSF; Urine	Medana IM, Day NP, Salahifar-Sabet H, Stocker R, Smythe G, Bwanaisa L, Njobvu A, Kayira K, Turner GD, Taylor TE, Hunt NH: Metabolites of the kynurenine pathway of tryptophan metabolism in the cerebrospinal fluid of Malawian children with malaria. J Infect Dis. 2003 Sep 15;188(6):844-9. Epub 2003 Sep 9. [PubMed ]
HMDB00070	Pipecolic acid	Amniotic Fluid; Blood; CSF; Urine	Medana IM, Day NP, Salahifar-Sabet H, Stocker R, Smythe G, Bwanaisa L, Njobvu A, Kayira K, Turner GD, Taylor TE, Hunt NH: Metabolites of the kynurenine pathway of tryptophan metabolism in the cerebrospinal fluid of Malawian children with malaria. J Infect Dis. 2003 Sep 15;188(6):844-9. Epub 2003 Sep 9. [PubMed ]
HMDB00232	Quinolinic acid	Blood; CSF; Urine	Medana IM, Day NP, Salahifar-Sabet H, Stocker R, Smythe G, Bwanaisa L, Njobvu A, Kayira K, Turner GD, Taylor TE, Hunt NH: Metabolites of the kynurenine pathway of tryptophan metabolism in the cerebrospinal fluid of Malawian children with malaria. J Infect Dis. 2003 Sep 15;188(6):844-9. Epub 2003 Sep 9. [PubMed ]

Coronary artery disease (2 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00195	Inosine	Blood; Cellular Cytoplasm; CSF; Pericardial Effusion; Urine	Fazekas L, Horkay F, Kekesi V, Huszar E, Barat E, Fazekas R, Szabo T, Juhasz-Nagy A, Naszlady A: Enhanced accumulation of pericardial fluid adenosine and inosine in patients with coronary artery disease. Life Sci. 1999;65(10):1005-12. [PubMed ]
HMDB04385	Lipoxin A4	Blood	Brezinski DA, Nesto RW, Serhan CN: Angioplasty triggers intracoronary leukotrienes and lipoxin A4. Impact of aspirin therapy. Circulation. 1992 Jul;86(1):56-63. [PubMed ]

Coronary heart disease (2 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB03125	Hydrogen peroxide	Blood	Kazmierczak M, Wysocki H, Wykretowicz A, Minczykowski A: Estimation of hydrogen peroxide plasma levels in patients evaluated for coronary heart disease using dipyridamole infusion followed by SPECT. Coron Artery Dis. 1995 Jan;6(1):65-9. [PubMed ]
HMDB01452	Thromboxane A2	Blood; Urine	Akimova EV: Prostacyclin and thromboxane A2 levels in children and adolescents with an inherited predisposition to coronary heart disease: a family study. Coron Artery Dis. 1994 Sep;5(9):761-5. [PubMed ]

Cresol poisoning (2 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB02048	m-Cresol	Blood; Urine	Monma-Ohtaki J, Maeno Y, Nagao M, Iwasa M, Koyama H, Isobe I, Seko-Nakamura Y, Tsuchimochi T, Matsumoto T: An autopsy case of poisoning by massive absorption of cresol a short time before death. Forensic Sci Int. 2002 Mar 28;126(1):77-81. [PubMed ]
HMDB02055	o-Cresol	Blood	Boatto G, Nieddu M, Carta A, Pau A, Lorenzoni S, Manconi P, Serra D: Determination of phenol and o-cresol by GC/MS in a fatal poisoning case. Forensic Sci Int. 2004 Jan 28;139(2-3):191-4. [PubMed ]

Crigler-Najjar syndrome Type I (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00054	Bilirubin	Bile; Blood; CSF; Urine	Metagene: CRIGLER-NAJJAR SYNDROME TYPE I

Critical illness (major trauma, severe septic shock, or cardiogenic shock) (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00195	Inosine	Blood; Cellular Cytoplasm; CSF; Pericardial Effusion; Urine	Jabs CM, Sigurdsson GH, Neglen P: Plasma levels of high-energy compounds compared with severity of illness in critically ill patients in the intensive care unit. Surgery. 1998 Jul;124(1):65-72. [PubMed ]

Crohn's disease (2 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00054	Bilirubin	Bile; Blood; CSF; Urine	Lapidus A, Akerlund JE, Einarsson C: Gallbladder bile composition in patients with Crohn 's disease. World J Gastroenterol. 2006 Jan 7;12(1):70-4. [PubMed ]
HMDB00098	D-Xylose	Blood; Urine	Ehrenpreis ED, Salvino M, Craig RM: Improving the serum D-xylose test for the identification of patients with small intestinal malabsorption. J Clin Gastroenterol. 2001 Jul;33(1):36-40. [PubMed ]

Cruetzfeldt-Jakob disease (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00742	Homocysteine	Blood; CSF; Urine	Bleich S, Otto M, Zerr I, Kropp S, Kretzschmar HA, Wiltfang J: Creutzfeldt-Jakob disease and homocysteine levels in plasma and cerebrospinal fluid. Gerontology. 2005 Mar-Apr;51(2):142-4. [PubMed ]

Cushing's syndrome (2 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB03955	19-Hydroxyandrost-4-ene-3,17-dione	Blood	Mune T, Morita H, Yasuda K, Murayama M, Yamakita N, Miura K: Elevated plasma 19-hydroxyandrostenedione levels in Cushing's disease: stimulation with ACTH and inhibition with metyrapone. Clin Endocrinol (Oxf). 1993 Mar;38(3):265-72. [PubMed ]
HMDB00053	Androstenedione	Blood; CSF; Urine	Mune T, Morita H, Yasuda K, Murayama M, Yamakita N, Miura K: Elevated plasma 19-hydroxyandrostenedione levels in Cushing's disease: stimulation with ACTH and inhibition with metyrapone. Clin Endocrinol (Oxf). 1993 Mar;38(3):265-72. [PubMed ]

Cystathionine beta-synthase deficiency (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00742	Homocysteine	Blood; CSF; Urine	Metagene: HOMOCYSTINURIA, CYSTATHIONINE BETA-SYNTHASE DEFICIENCY

Cystathioninuria (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00099	L-Cystathionine	Blood; CSF; Urine	Sugahara K, Ohta J, Takemura M, Kodama H: Determination of cystathionine and perhydro-1,4-thiazepine-3,5-dicarboxylic acid in the urine of a patient with cystathioninuria using column liquid chromatography-mass spectrometry. J Chromatogr. 1992 Sep 2;579(2):318-23. [PubMed ]

Cystic fibrosis (6 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00518	Chenodeoxycholic acid	Bile; Blood; Urine	Smith JL, Lewindon PJ, Hoskins AC, Pereira TN, Setchell KD, O'Connell NC, Shepherd RW, Ramm GA: Endogenous ursodeoxycholic acid and cholic acid in liver disease due to cystic fibrosis. Hepatology. 2004 Jun;39(6):1673-82. [PubMed ]
HMDB00619	Cholic acid	Bile; Blood; Urine	Smith JL, Lewindon PJ, Hoskins AC, Pereira TN, Setchell KD, O'Connell NC, Shepherd RW, Ramm GA: Endogenous ursodeoxycholic acid and cholic acid in liver disease due to cystic fibrosis. Hepatology. 2004 Jun;39(6):1673-82. [PubMed ]
HMDB00626	Deoxycholic acid	Bile; Blood; Urine	Smith JL, Lewindon PJ, Hoskins AC, Pereira TN, Setchell KD, O'Connell NC, Shepherd RW, Ramm GA: Endogenous ursodeoxycholic acid and cholic acid in liver disease due to cystic fibrosis. Hepatology. 2004 Jun;39(6):1673-82. [PubMed ]
HMDB00138	Glycocholic acid	Blood; Urine	Matsui A, Psacharopoulos HT, Mowat AP, Portmann B, Murphy GM: Radioimmunoassay of serum glycocholic acid, standard laboratory tests of liver function and liver biopsy findings: comparative study of children with liver disease. J Clin Pathol. 1982 Sep;35(9):1011-7. [PubMed ]
HMDB00761	Lithocholic acid	Bile; Blood; Urine	Smith JL, Lewindon PJ, Hoskins AC, Pereira TN, Setchell KD, O'Connell NC, Shepherd RW, Ramm GA: Endogenous ursodeoxycholic acid and cholic acid in liver disease due to cystic fibrosis. Hepatology. 2004 Jun;39(6):1673-82. [PubMed ]
HMDB00946	Ursodeoxycholic acid	Blood; Urine	Smith JL, Lewindon PJ, Hoskins AC, Pereira TN, Setchell KD, O'Connell NC, Shepherd RW, Ramm GA: Endogenous ursodeoxycholic acid and cholic acid in liver disease due to cystic fibrosis. Hepatology. 2004 Jun;39(6):1673-82. [PubMed ]

Cystinosis (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00067	Cholesterol	Bile; Blood; CSF; Saliva; Urine	Metagene: CYSTINOSIS

Cystinuria (4 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00517	L-Arginine	Blood; CSF; Urine	Metagene: CYSTINURIA
HMDB00192	L-Cystine	Blood; CSF; Urine	Metagene: CYSTINURIA
HMDB00182	L-Lysine	Blood; CSF; Saliva; Urine	Metagene: CYSTINURIA
HMDB00214	Ornithine	Blood; CSF; Saliva; Urine	Metagene: CYSTINURIA

Cytochrome C oxidase deficiency (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00765	Mannitol	Blood; CSF; Urine	Shetty HU, Holloway HW, Rapoport SI: Capillary gas chromatography combined with ion trap detection for quantitative profiling of polyols in cerebrospinal fluid and plasma. Anal Biochem. 1995 Jan 1;224(1):279-85. [PubMed ]

Cytochrome-C-Oxidase deficiency (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00190	L-Lactic acid	Bile; Blood; Cellular Cytoplasm; CSF; Urine	Metagene: CYTOCHROME-C-OXIDASE DEFICIENCY

D-2-Hydroxyglutaric aciduria (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00606	D-2-Hydroxyglutaric acid	Blood; CSF; Urine	Gibson KM, ten Brink HJ, Schor DS, Kok RM, Bootsma AH, Hoffmann GF, Jakobs C: Stable-isotope dilution analysis of D- and L-2-hydroxyglutaric acid: application to the detection and prenatal diagnosis of D- and L-2-hydroxyglutaric acidemias. Pediatr Res. 1993 Sep;34(3):277-80. [PubMed ]

D-Glyceric acidura (2 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00139	Glyceric acid	Blood; CSF; N/A; Urine	Metagene: D-GLYCERIC ACIDURA
HMDB00123	Glycine	Bile; Blood; CSF; Saliva; Urine	Metagene: D-GLYCERIC ACIDURA

Deficiency in the 11 beta-hydroxylation of corticosteroids (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00879	Tetrahydrodeoxycorticosterone	Blood; Urine	Cathelineau G, Brerault JL, Fiet J, Julien R, Dreux C, Canivet J: Adrenocortical 11 beta-hydroxylation defect in adult women with postmenarchial onset of symptoms. J Clin Endocrinol Metab. 1980 Aug;51(2):287-91. [PubMed ]

Degenerative disc disease (6 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00157	Hypoxanthine	Amniotic Fluid; Blood; Cellular Cytoplasm; CSF; Urine	Eells JT, Spector R: Purine and pyrimidine base and nucleoside concentrations in human cerebrospinal fluid and plasma. Neurochem Res. 1983 Nov;8(11):1451-7. [PubMed ]
HMDB00195	Inosine	Blood; Cellular Cytoplasm; CSF; Pericardial Effusion; Urine	Eells JT, Spector R: Purine and pyrimidine base and nucleoside concentrations in human cerebrospinal fluid and plasma. Neurochem Res. 1983 Nov;8(11):1451-7. [PubMed ]
HMDB00273	Thymidine	Amniotic Fluid; Blood; CSF; Urine	Eells JT, Spector R: Purine and pyrimidine base and nucleoside concentrations in human cerebrospinal fluid and plasma. Neurochem Res. 1983 Nov;8(11):1451-7. [PubMed ]
HMDB00289	Uric acid	Amniotic Fluid; Bile; Blood; CSF; Saliva; Urine	Eells JT, Spector R: Purine and pyrimidine base and nucleoside concentrations in human cerebrospinal fluid and plasma. Neurochem Res. 1983 Nov;8(11):1451-7. [PubMed ]
HMDB00296	Uridine	Blood; CSF; Urine	Eells JT, Spector R: Purine and pyrimidine base and nucleoside concentrations in human cerebrospinal fluid and plasma. Neurochem Res. 1983 Nov;8(11):1451-7. [PubMed ]
HMDB00292	Xanthine	Blood; CSF; Urine	Eells JT, Spector R: Purine and pyrimidine base and nucleoside concentrations in human cerebrospinal fluid and plasma. Neurochem Res. 1983 Nov;8(11):1451-7. [PubMed ]

Dementia (Alzheimer's and non-Alzheimer's) (2 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00742	Homocysteine	Blood; CSF; Urine	Obeid R, Kasoha M, Knapp JP, Kostopoulos P, Becker G, Fassbender K, Herrmann W: Folate and methylation status in relation to phosphorylated tau protein(181P) and beta-amyloid(1-42) in cerebrospinal fluid. Clin Chem. 2007 Jun;53(6):1129-36. Epub 2007 Mar 23. [PubMed ]
HMDB00574	L-Cysteine	Blood; CSF; Urine	Obeid R, Kasoha M, Knapp JP, Kostopoulos P, Becker G, Fassbender K, Herrmann W: Folate and methylation status in relation to phosphorylated tau protein(181P) and beta-amyloid(1-42) in cerebrospinal fluid. Clin Chem. 2007 Jun;53(6):1129-36. Epub 2007 Mar 23. [PubMed ]

Dementia - unspecified cause (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB04207	L-a-glutamyl-L-Lysine	CSF	Jeitner TM, Bogdanov MB, Matson WR, Daikhin Y, Yudkoff M, Folk JE, Steinman L, Browne SE, Beal MF, Blass JP, Cooper AJ: N(epsilon)-(gamma-L-glutamyl)-L-lysine (GGEL) is increased in cerebrospinal fluid of patients with Huntington's disease. J Neurochem. 2001 Dec;79(5):1109-12. [PubMed ]

Dementia of Alzheimer type (DAT) (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00190	L-Lactic acid	Bile; Blood; Cellular Cytoplasm; CSF; Urine	Redjems-Bennani N, Jeandel C, Lefebvre E, Blain H, Vidailhet M, Gueant JL: Abnormal substrate levels that depend upon mitochondrial function in cerebrospinal fluid from Alzheimer patients. Gerontology. 1998;44(5):300-4. [PubMed ]

Dengue fever (3 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00161	L-Alanine	Blood; CSF; Saliva; Urine	Klassen P, Furst P, Schulz C, Mazariegos M, Solomons NW: Plasma free amino acid concentrations in healthy Guatemalan adults and in patients with classic dengue. Am J Clin Nutr. 2001 Mar;73(3):647-52. [PubMed ]
HMDB00177	L-Histidine	Blood; CSF; Saliva; Urine	Klassen P, Furst P, Schulz C, Mazariegos M, Solomons NW: Plasma free amino acid concentrations in healthy Guatemalan adults and in patients with classic dengue. Am J Clin Nutr. 2001 Mar;73(3):647-52. [PubMed ]
HMDB00159	L-Phenylalanine	Blood; CSF; Saliva; Urine	Klassen P, Furst P, Schulz C, Mazariegos M, Solomons NW: Plasma free amino acid concentrations in healthy Guatemalan adults and in patients with classic dengue. Am J Clin Nutr. 2001 Mar;73(3):647-52. [PubMed ]

Depersonalization syndrome (2 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB01547	Corticosterone	Blood	Nuller YL, Morozova MG, Kushnir ON, Hamper N: Effect of naloxone therapy on depersonalization: a pilot study. J Psychopharmacol. 2001 Jun;15(2):93-5. [PubMed ]
HMDB02802	Cortisone	Blood; Urine	Nuller YL, Morozova MG, Kushnir ON, Hamper N: Effect of naloxone therapy on depersonalization: a pilot study. J Psychopharmacol. 2001 Jun;15(2):93-5. [PubMed ]

Dermal fibroproliferative disorder (hypertrophic scarring) (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00898	1-Methylhistamine	Blood; CSF; Urine	Tredget EE, Iwashina T, Scott PG, Ghahary A: Determination of plasma Ntau-methylhistamine in vivo by isotope dilution using benchtop gas chromatography-mass spectrometry. J Chromatogr B Biomed Sci Appl. 1997 Jun 20;694(1):1-9. [PubMed ]

Diabetes (27 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00011	(R)-3-Hydroxybutyric acid	Blood; CSF; Urine	Sheikh-Ali M, Karon BS, Basu A, Kudva YC, Muller LA, Xu J, Schwenk WF, Miles JM: Can serum beta-hydroxybutyrate be used to diagnose diabetic ketoacidosis? Diabetes Care. 2008 Apr;31(4):643-7. Epub 2008 Jan 9. [PubMed ]
HMDB00336	(R)-3-Hydroxyisobutyric acid	Blood; CSF	Avogaro A, Bier DM: Contribution of 3-hydroxyisobutyrate to the measurement of 3-hydroxybutyrate in human plasma: comparison of enzymatic and gas-liquid chromatography-mass spectrometry assays in normal and in diabetic subjects. J Lipid Res. 1989 Nov;30(11):1811-7. [PubMed ]
HMDB00023	(S)-3-Hydroxyisobutyric acid	Blood; CSF; Urine	Avogaro A, Bier DM: Contribution of 3-hydroxyisobutyrate to the measurement of 3-hydroxybutyrate in human plasma: comparison of enzymatic and gas-liquid chromatography-mass spectrometry assays in normal and in diabetic subjects. J Lipid Res. 1989 Nov;30(11):1811-7. [PubMed ]
HMDB02712	1,5-Anhydrosorbitol	Blood; CSF	Nguyen TM, Rodriguez LM, Mason KJ, Heptulla RA: Serum 1,5-anhydroglucitol (Glycomark) levels in children with and without type 1 diabetes mellitus. Pediatr Diabetes. 2007 Aug;8(4):214-9. [PubMed ]
HMDB04327	1-Butanol	Blood	Liebich HM, Buelow HJ, Kallmayer R: Quantification of endogenous aliphatic alcohols in serum and urine. J Chromatogr. 1982 Apr 30;239:343-9. [PubMed ]
HMDB00001	1-Methylhistidine	Blood; CSF; Saliva; Urine	Tuma P, Samcova E, Balinova P: Determination of 3-methylhistidine and 1-methylhistidine in untreated urine samples by capillary electrophoresis. J Chromatogr B Analyt Technol Biomed Life Sci. 2005 Jul 5;821(1):53-9. [PubMed ]
HMDB00357	3-Hydroxybutyric acid	Blood; Cellular Cytoplasm; CSF; Urine	del Carmen Crespillo M, Olveira G, de Adana MS, Rojo-Martinez G, Garcia-Aleman J, Olvera P, Soriguer F, Munoz A: Metabolic effects of an enteral nutrition formula for diabetes: comparison with standard formulas in patients with type 1 diabetes. Clin Nutr. 2003 Oct;22(5):483-7. [PubMed ]
HMDB04814	4-Heptanone	Blood; Urine	Wahl HG, Hoffmann A, Luft D, Liebich HM: Analysis of volatile organic compounds in human urine by headspace gas chromatography-mass spectrometry with a multipurpose sampler. J Chromatogr A. 1999 Jun 25;847(1-2):117-25. [PubMed ]
HMDB02032	8-Hydroxyguanine	Blood; CSF	Shin CS, Moon BS, Park KS, Kim SY, Park SJ, Chung MH, Lee HK: Serum 8-hydroxy-guanine levels are increased in diabetic patients. Diabetes Care. 2001 Apr;24(4):733-7. [PubMed ]
HMDB00060	Acetoacetic acid	Blood; Cellular Cytoplasm; CSF; Urine	Tasker RC, Lutman D, Peters MJ: Hyperventilation in severe diabetic ketoacidosis. Pediatr Crit Care Med. 2005 Jul;6(4):405-11. [PubMed ]
HMDB01659	Acetone	Blood; CSF; Urine	Bales JR, Higham DP, Howe I, Nicholson JK, Sadler PJ: Use of high-resolution proton nuclear magnetic resonance spectroscopy for rapid multi-component analysis of urine. Clin Chem. 1984 Mar;30(3):426-32. [PubMed ]
HMDB00599	Chromium	Blood; CSF; Urine	Stupar J, Vrtovec M, Dolinsek F: Longitudinal hair chromium profiles of elderly subjects with normal glucose tolerance and type 2 diabetes mellitus. Metabolism. 2007 Jan;56(1):94-104. [PubMed ]
HMDB00660	D-Fructose	Blood; CSF; Urine	Kawasaki T, Akanuma H, Yamanouchi T: Increased fructose concentrations in blood and urine in patients with diabetes. Diabetes Care. 2002 Feb;25(2):353-7. [PubMed ]
HMDB00122	D-Glucose	Blood; Breast Milk; CSF; Saliva; Urine	Bales JR, Higham DP, Howe I, Nicholson JK, Sadler PJ: Use of high-resolution proton nuclear magnetic resonance spectroscopy for rapid multi-component analysis of urine. Clin Chem. 1984 Mar;30(3):426-32. [PubMed ]
HMDB01311	D-Lactic acid	Blood; Urine	McLellan AC, Phillips SA, Thornalley PJ: Fluorimetric assay of D-lactate. Anal Biochem. 1992 Oct;206(1):12-6. [PubMed ]
HMDB00087	Dimethylamine	Blood; CSF; Saliva; Urine	Zhang AQ, Mitchell SC, Smith RL: Dimethylamine in human urine. Clin Chim Acta. 1995 Jan 16;233(1-2):81-8. [PubMed ]
HMDB00623	Dodecanedioic acid	Blood	Greco AV, Mingrone G, Capristo E, Benedetti G, De Gaetano A, Gasbarrini G: The metabolic effect of dodecanedioic acid infusion in non-insulin-dependent diabetic patients. Nutrition. 1998 Apr;14(4):351-7. [PubMed ]
HMDB00153	Estriol	Amniotic Fluid; Blood; Urine	Giussi GA, Ballejo G, Benedetti WL: Estriol serum levels, neonatal vitality and stromal villous edema in diabetic pregnancies. Acta Diabetol Lat. 1979 Apr-Jun;16(2):105-10. [PubMed ]
HMDB02030	Fructosamine	Blood; Saliva	Guevara-Aguirre J, Guevara-Aguirre M, Saavedra J, Bernstein G, Rosenbloom AL: Comparison of oral insulin spray and subcutaneous regular insulin at mealtime in type 1 diabetes. Diabetes Technol Ther. 2007 Aug;9(4):372-6. [PubMed ]
HMDB00131	Glycerol	Blood; CSF; Urine	Blomqvist G, Alvarsson M, Grill V, Von Heijne G, Ingvar M, Thorell JO, Stone-Elander S, Widen L, Ekberg K: Effect of acute hyperketonemia on the cerebral uptake of ketone bodies in nondiabetic subjects and IDDM patients. Am J Physiol Endocrinol Metab. 2002 Jul;283(1):E20-8. [PubMed ]
HMDB10366	Hyaluronan	Blood	Mine S, Okada Y, Kawahara C, Tabata T, Tanaka Y: Serum hyaluronan concentration as a marker of angiopathy in patients with diabetes mellitus. Endocr J. 2006 Dec;53(6):761-6. Epub 2006 Sep 12. [PubMed ]
HMDB00062	L-Carnitine	Blood; CSF; Urine	Hoppel CL, Genuth SM: Urinary excretion of acetylcarnitine during human diabetic and fasting ketosis. Am J Physiol. 1982 Aug;243(2):E168-72. [PubMed ]
HMDB03378	Nitric oxide	Blood; CSF; Urine	Savino A, Pelliccia P, Schiavone C, Primavera A, Tumini S, Mohn A, Chiarelli F: Serum and urinary nitrites and nitrates and Doppler sonography in children with diabetes. Diabetes Care. 2006 Dec;29(12):2676-81. [PubMed ]
HMDB01167	Pyruvaldehyde	Blood; Urine	Khuhawar MY, Zardari LA, Laghari AJ: Capillary gas chromatographic determination of methylglyoxal from serum of diabetic patients by precolumn derivatization with 1,2-diamonopropane. J Chromatogr B Analyt Technol Biomed Life Sci. 2008 Sep 15;873(1):15-9. Epub 2008 May 17. [PubMed ]
HMDB01185	S-Adenosylmethionine	Blood; CSF; Urine	Hoppel CL, Genuth SM: Urinary excretion of acetylcarnitine during human diabetic and fasting ketosis. Am J Physiol. 1982 Aug;243(2):E168-72. [PubMed ]
HMDB06088	Scyllitol	Blood; CSF	Ostlund RE Jr, McGill JB, Herskowitz I, Kipnis DM, Santiago JV, Sherman WR: D-chiro-inositol metabolism in diabetes mellitus. Proc Natl Acad Sci U S A. 1993 Nov 1;90(21):9988-92. [PubMed ]
HMDB00289	Uric acid	Amniotic Fluid; Bile; Blood; CSF; Saliva; Urine	Costa A, Iguala I, Bedini J, Quinto L, Conget I: Uric acid concentration in subjects at risk of type 2 diabetes mellitus: relationship to components of the metabolic syndrome. Metabolism. 2002 Mar;51(3):372-5. [PubMed ]

Diabetes mellitus (2 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00479	3-Methylhistidine	Blood; CSF; Urine	Tuma P, Samcova E, Balinova P: Determination of 3-methylhistidine and 1-methylhistidine in untreated urine samples by capillary electrophoresis. J Chromatogr B Analyt Technol Biomed Life Sci. 2005 Jul 5;821(1):53-9. [PubMed ]
HMDB00122	D-Glucose	Blood; Breast Milk; CSF; Saliva; Urine	World Health Organisation Department of Noncommunicable Disease Surveillance (1999). "Definition, Diagnosis and Classification of Diabetes Mellitus and its Complications"

Diabetic ketoacidosis (2 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00357	3-Hydroxybutyric acid	Blood; Cellular Cytoplasm; CSF; Urine	Charles RA, Bee YM, Eng PH, Goh SY: Point-of-care blood ketone testing: screening for diabetic ketoacidosis at the emergency department. Singapore Med J. 2007 Nov;48(11):986-9. [PubMed ]
HMDB00595	Hydrogen carbonate	Blood; Cellular Cytoplasm; CSF	Gill GV, MacNamara G, English P: Diabetic ketoacidosis complicated by axillary vein thrombosis. Diabetes Res Clin Pract. 2006 Jul;73(1):104-6. Epub 2006 Jan 18. [PubMed ]

Dihydropyrimidinase deficiency (2 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00079	Dihydrothymine	CSF; Urine	van Kuilenburg AB, Meijer J, Dobritzsch D, Meinsma R, Duran M, Lohkamp B, Zoetekouw L, Abeling NG, van Tinteren HL, Bosch AM: Clinical, biochemical and genetic findings in two siblings with a dihydropyrimidinase deficiency. Mol Genet Metab. 2007 Jun;91(2):157-64. Epub 2007 Mar 26. [PubMed ]
HMDB00026	Ureidopropionic acid	CSF; Urine	van Kuilenburg AB, Meijer J, Dobritzsch D, Meinsma R, Duran M, Lohkamp B, Zoetekouw L, Abeling NG, van Tinteren HL, Bosch AM: Clinical, biochemical and genetic findings in two siblings with a dihydropyrimidinase deficiency. Mol Genet Metab. 2007 Jun;91(2):157-64. Epub 2007 Mar 26. [PubMed ]

Dihydropyrimidine dehydrogenase (DPD) deficiency (2 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00056	Beta-Alanine	Blood; CSF; Urine	Van Kuilenburg AB, Stroomer AE, Van Lenthe H, Abeling NG, Van Gennip AH: New insights in dihydropyrimidine dehydrogenase deficiency: a pivotal role for beta-aminoisobutyric acid? Biochem J. 2004 Apr 1;379(Pt 1):119-24. [PubMed ]
HMDB00076	Dihydrouracil	Blood; CSF; Urine	Sumi S, Kidouchi K, Ohba S, Wada Y: Automated screening system for purine and pyrimidine metabolism disorders using high-performance liquid chromatography. J Chromatogr B Biomed Appl. 1995 Oct 20;672(2):233-9. [PubMed ]

Dihydropyrimidine dehydrogenase deficiency (2 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00262	Thymine	Blood; CSF; Urine	Metagene: DIHYDROPYRIMIDINE DEHYDROGENASE DEFICIENCY (DHPD)
HMDB00300	Uracil	Amniotic Fluid; Blood; CSF; Urine	Metagene: DIHYDROPYRIMIDINE DEHYDROGENASE DEFICIENCY (DHPD)

Dimethyl sulfide poisoning (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB02303	Dimethylsulfide	Blood; CSF	Terazawa K, Kaji H, Akabane H, Takatori T: Determination of dimethyl sulphide in blood and adipose tissue by headspace gas analysis. J Chromatogr. 1991 Apr 19;565(1-2):453-6. [PubMed ]

Dimethyl sulfone supplementation (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB04983	Dimethyl sulfone	Blood; CSF; Urine	Engelke UF, Tangerman A, Willemsen MA, Moskau D, Loss S, Mudd SH, Wevers RA: Dimethyl sulfone in human cerebrospinal fluid and blood plasma confirmed by one-dimensional (1)H and two-dimensional (1)H-(13)C NMR. NMR Biomed. 2005 Aug;18(5):331-6. [PubMed ]

Down's syndrome (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00118	Homovanillic acid	Blood; CSF; Urine	Kay AD, Schapiro MB, Riker AK, Haxby JV, Rapoport SI, Cutler NR: Cerebrospinal fluid monoaminergic metabolites are elevated in adults with Down's syndrome. Ann Neurol. 1987 Apr;21(4):408-11. [PubMed ]

Drunk driver (2 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00108	Ethanol	Blood; CSF; Saliva; Urine	Jones AW, Helander A: Changes in the concentrations of ethanol, methanol and metabolites of serotonin in two successive urinary voids from drinking drivers. Forensic Sci Int. 1998 May 11;93(2-3):127-34. [PubMed ]
HMDB01875	Methanol	Blood; CSF; Saliva; Urine	Jones AW, Helander A: Changes in the concentrations of ethanol, methanol and metabolites of serotonin in two successive urinary voids from drinking drivers. Forensic Sci Int. 1998 May 11;93(2-3):127-34. [PubMed ]

Early-onset encephalopathy and cortical myoclonus (MECP2 gene mutation) (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB01490	Vanylglycol	Blood; CSF; Urine	Leuzzi V, Di Sabato ML, Zollino M, Montanaro ML, Seri S: Early-onset encephalopathy and cortical myoclonus in a boy with MECP2 gene mutation. Neurology. 2004 Nov 23;63(10):1968-70. [PubMed ]

Eating disorder (5 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00526	5a-Tetrahydrocortisol	Blood; Urine	Poor V, Biro I, Bufa A, Gati A, Fenyvesi I, Juricskay S, Tenyi T, Kilar F: Urinary steroids in young women with eating disorders. J Biochem Biophys Methods. 2004 Oct 29;61(1-2):199-205. [PubMed ]
HMDB03180	Cortol	Urine	Poor V, Biro I, Bufa A, Gati A, Fenyvesi I, Juricskay S, Tenyi T, Kilar F: Urinary steroids in young women with eating disorders. J Biochem Biophys Methods. 2004 Oct 29;61(1-2):199-205. [PubMed ]
HMDB03128	Cortolone	Urine	Poor V, Biro I, Bufa A, Gati A, Fenyvesi I, Juricskay S, Tenyi T, Kilar F: Urinary steroids in young women with eating disorders. J Biochem Biophys Methods. 2004 Oct 29;61(1-2):199-205. [PubMed ]
HMDB00949	Tetrahydrocortisol	Urine	Poor V, Biro I, Bufa A, Gati A, Fenyvesi I, Juricskay S, Tenyi T, Kilar F: Urinary steroids in young women with eating disorders. J Biochem Biophys Methods. 2004 Oct 29;61(1-2):199-205. [PubMed ]
HMDB00903	Tetrahydrocortisone	Blood; Urine	Poor V, Biro I, Bufa A, Gati A, Fenyvesi I, Juricskay S, Tenyi T, Kilar F: Urinary steroids in young women with eating disorders. J Biochem Biophys Methods. 2004 Oct 29;61(1-2):199-205. [PubMed ]

Eczema (2 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB01198	Leukotriene C4	Blood	Hua Z, Fei H, Mingming X: Evaluation and interference of serum and skin lesion levels of leukotrienes in patients with eczema. Prostaglandins Leukot Essent Fatty Acids. 2006 Jul;75(1):51-5. Epub 2006 Jun 6. [PubMed ]
HMDB02200	Leukotriene E4	Blood; Urine	Hua Z, Fei H, Mingming X: Evaluation and interference of serum and skin lesion levels of leukotrienes in patients with eczema. Prostaglandins Leukot Essent Fatty Acids. 2006 Jul;75(1):51-5. Epub 2006 Jun 6. [PubMed ]

Encephalitis (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB01336	3,4-Dihydroxybenzeneacetic acid	Blood; CSF; Urine	Kalita J, Kumar S, Vijaykumar K, Palit G, Misra UK: A study of CSF catecholamine and its metabolites in acute and convalescent period of encephalitis. J Neurol Sci. 2007 Jan 15;252(1):62-6. Epub 2006 Nov 28. [PubMed ]

Endometrial cancer (3 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00561	B-Carotene	Blood	Jeong NH, Song ES, Lee JM, Lee KB, Kim MK, Yun YM, Lee JK, Son SK, Lee JP, Kim JH, Hur SY, Kwon YI: Preoperative levels of plasma micronutrients are related to endometrial cancer risk. Acta Obstet Gynecol Scand. 2009;88(4):434-9. [PubMed ]
HMDB01492	Gama-Tocopherol	Blood; Breast Milk	Jeong NH, Song ES, Lee JM, Lee KB, Kim MK, Yun YM, Lee JK, Son SK, Lee JP, Kim JH, Hur SY, Kwon YI: Preoperative levels of plasma micronutrients are related to endometrial cancer risk. Acta Obstet Gynecol Scand. 2009;88(4):434-9. [PubMed ]
HMDB03000	Lycopene	Blood	Jeong NH, Song ES, Lee JM, Lee KB, Kim MK, Yun YM, Lee JK, Son SK, Lee JP, Kim JH, Hur SY, Kwon YI: Preoperative levels of plasma micronutrients are related to endometrial cancer risk. Acta Obstet Gynecol Scand. 2009;88(4):434-9. [PubMed ]

Epilepsy (26 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00763	5-Hydroxyindoleacetic acid	Blood; CSF; Urine	Shaywitz BA, Cohen DJ, Bowers MB: Reduced cerebrospinal fluid 5-hydroxyindoleacetic acid and homovanillic acid in children with epilepsy. Neurology. 1975 Jan;25(1):72-9. [PubMed ]
HMDB00538	Adenosine triphosphate	Blood; Cellular Cytoplasm; CSF	Czarnecka J, Cieslak M, Michal K: Application of solid phase extraction and high-performance liquid chromatography to qualitative and quantitative analysis of nucleotides and nucleosides in human cerebrospinal fluid. J Chromatogr B Analyt Technol Biomed Life Sci. 2005 Aug 5;822(1-2):85-90. [PubMed ]
HMDB01341	ADP	Blood; Cellular Cytoplasm; CSF	Czarnecka J, Cieslak M, Michal K: Application of solid phase extraction and high-performance liquid chromatography to qualitative and quantitative analysis of nucleotides and nucleosides in human cerebrospinal fluid. J Chromatogr B Analyt Technol Biomed Life Sci. 2005 Aug 5;822(1-2):85-90. [PubMed ]
HMDB00904	Citrulline	Blood; CSF; Urine	Rainesalo S, Keranen T, Palmio J, Peltola J, Oja SS, Saransaari P: Plasma and cerebrospinal fluid amino acids in epileptic patients. Neurochem Res. 2004 Jan;29(1):319-24. [PubMed ]
HMDB00112	Gamma-Aminobutyric acid	Blood; CSF; Saliva; Urine	Spanaki MV, Siegel H, Kopylev L, Fazilat S, Dean A, Liow K, Ben-Menachem E, Gaillard WD, Theodore WH: The effect of vigabatrin (gamma-vinyl GABA) on cerebral blood flow and metabolism. Neurology. 1999 Oct 22;53(7):1518-22. [PubMed ]
HMDB01201	Guanosine diphosphate	Blood; CSF	Czarnecka J, Cieslak M, Michal K: Application of solid phase extraction and high-performance liquid chromatography to qualitative and quantitative analysis of nucleotides and nucleosides in human cerebrospinal fluid. J Chromatogr B Analyt Technol Biomed Life Sci. 2005 Aug 5;822(1-2):85-90. [PubMed ]
HMDB01273	Guanosine triphosphate	Blood; CSF	Czarnecka J, Cieslak M, Michal K: Application of solid phase extraction and high-performance liquid chromatography to qualitative and quantitative analysis of nucleotides and nucleosides in human cerebrospinal fluid. J Chromatogr B Analyt Technol Biomed Life Sci. 2005 Aug 5;822(1-2):85-90. [PubMed ]
HMDB00118	Homovanillic acid	Blood; CSF; Urine	Shaywitz BA, Cohen DJ, Bowers MB: Reduced cerebrospinal fluid 5-hydroxyindoleacetic acid and homovanillic acid in children with epilepsy. Neurology. 1975 Jan;25(1):72-9. [PubMed ]
HMDB02061	Hyaluronic acid	Blood; CSF	Perosa SR, Porcionatto MA, Cukiert A, Martins JR, Amado D, Nader HB, Cavalheiro EA, Leite JP, Naffah-Mazzacoratti MG: Extracellular matrix components are altered in the hippocampus, cortex, and cerebrospinal fluid of patients with mesial temporal lobe epilepsy. Epilepsia. 2002;43 Suppl 5:159-61. [PubMed ]
HMDB00161	L-Alanine	Blood; CSF; Saliva; Urine	Rainesalo S, Keranen T, Palmio J, Peltola J, Oja SS, Saransaari P: Plasma and cerebrospinal fluid amino acids in epileptic patients. Neurochem Res. 2004 Jan;29(1):319-24. [PubMed ]
HMDB00168	L-Asparagine	Blood; CSF; Urine	Rainesalo S, Keranen T, Palmio J, Peltola J, Oja SS, Saransaari P: Plasma and cerebrospinal fluid amino acids in epileptic patients. Neurochem Res. 2004 Jan;29(1):319-24. [PubMed ]
HMDB00191	L-Aspartic acid	Blood; CSF; Saliva; Urine	Rainesalo S, Keranen T, Palmio J, Peltola J, Oja SS, Saransaari P: Plasma and cerebrospinal fluid amino acids in epileptic patients. Neurochem Res. 2004 Jan;29(1):319-24. [PubMed ]
HMDB00148	L-Glutamic acid	Blood; Cellular Cytoplasm; CSF; Saliva; Urine	Rainesalo S, Keranen T, Palmio J, Peltola J, Oja SS, Saransaari P: Plasma and cerebrospinal fluid amino acids in epileptic patients. Neurochem Res. 2004 Jan;29(1):319-24. [PubMed ]
HMDB00641	L-Glutamine	Blood; CSF; Saliva; Urine	Rainesalo S, Keranen T, Palmio J, Peltola J, Oja SS, Saransaari P: Plasma and cerebrospinal fluid amino acids in epileptic patients. Neurochem Res. 2004 Jan;29(1):319-24. [PubMed ]
HMDB00177	L-Histidine	Blood; CSF; Saliva; Urine	Rainesalo S, Keranen T, Palmio J, Peltola J, Oja SS, Saransaari P: Plasma and cerebrospinal fluid amino acids in epileptic patients. Neurochem Res. 2004 Jan;29(1):319-24. [PubMed ]
HMDB00172	L-Isoleucine	Blood; CSF; Saliva; Urine	Rainesalo S, Keranen T, Palmio J, Peltola J, Oja SS, Saransaari P: Plasma and cerebrospinal fluid amino acids in epileptic patients. Neurochem Res. 2004 Jan;29(1):319-24. [PubMed ]
HMDB00687	L-Leucine	Blood; CSF; Urine	Rainesalo S, Keranen T, Palmio J, Peltola J, Oja SS, Saransaari P: Plasma and cerebrospinal fluid amino acids in epileptic patients. Neurochem Res. 2004 Jan;29(1):319-24. [PubMed ]
HMDB00696	L-Methionine	Blood; CSF; Urine	Rainesalo S, Keranen T, Palmio J, Peltola J, Oja SS, Saransaari P: Plasma and cerebrospinal fluid amino acids in epileptic patients. Neurochem Res. 2004 Jan;29(1):319-24. [PubMed ]
HMDB00159	L-Phenylalanine	Blood; CSF; Saliva; Urine	Rainesalo S, Keranen T, Palmio J, Peltola J, Oja SS, Saransaari P: Plasma and cerebrospinal fluid amino acids in epileptic patients. Neurochem Res. 2004 Jan;29(1):319-24. [PubMed ]
HMDB00167	L-Threonine	Blood; CSF; Saliva; Urine	Rainesalo S, Keranen T, Palmio J, Peltola J, Oja SS, Saransaari P: Plasma and cerebrospinal fluid amino acids in epileptic patients. Neurochem Res. 2004 Jan;29(1):319-24. [PubMed ]
HMDB00929	L-Tryptophan	Blood; CSF; Saliva; Urine	Rainesalo S, Keranen T, Palmio J, Peltola J, Oja SS, Saransaari P: Plasma and cerebrospinal fluid amino acids in epileptic patients. Neurochem Res. 2004 Jan;29(1):319-24. [PubMed ]
HMDB00158	L-Tyrosine	Blood; CSF; Saliva; Urine	Rainesalo S, Keranen T, Palmio J, Peltola J, Oja SS, Saransaari P: Plasma and cerebrospinal fluid amino acids in epileptic patients. Neurochem Res. 2004 Jan;29(1):319-24. [PubMed ]
HMDB00883	L-Valine	Blood; CSF; Saliva; Urine	Rainesalo S, Keranen T, Palmio J, Peltola J, Oja SS, Saransaari P: Plasma and cerebrospinal fluid amino acids in epileptic patients. Neurochem Res. 2004 Jan;29(1):319-24. [PubMed ]
HMDB00020	p-Hydroxyphenylacetic acid	Blood; CSF; Saliva; Urine	Kobayashi K, Koide Y, Yoshino K, Shohmori T: [P-hydroxyphenylacetic acid concentrations in cerebrospinal fluid] No To Shinkei. 1982 Aug;34(8):769-74. [PubMed ]
HMDB00251	Taurine	Bile; Blood; CSF; Urine	Rainesalo S, Keranen T, Palmio J, Peltola J, Oja SS, Saransaari P: Plasma and cerebrospinal fluid amino acids in epileptic patients. Neurochem Res. 2004 Jan;29(1):319-24. [PubMed ]
HMDB01490	Vanylglycol	Blood; CSF; Urine	Botez MI, Young SN: Effects of anticonvulsant treatment and low levels of folate and thiamine on amine metabolites in cerebrospinal fluid. Brain. 1991 Feb;114 ( Pt 1A):333-48. [PubMed ]

Essential hypertension (5 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB01388	Alpha-Linolenic acid	Blood; Urine	Wang S, Ma A, Song S, Quan Q, Zhao X, Zheng X: Fasting serum free fatty acid composition, waist/hip ratio and insulin activity in essential hypertensive patients. Hypertens Res. 2008 Apr;31(4):623-32. [PubMed ]
HMDB01043	Arachidonic acid	Blood; CSF; Urine	Wang S, Ma A, Song S, Quan Q, Zhao X, Zheng X: Fasting serum free fatty acid composition, waist/hip ratio and insulin activity in essential hypertensive patients. Hypertens Res. 2008 Apr;31(4):623-32. [PubMed ]
HMDB02183	Docosahexaenoic acid	Blood; CSF; Urine	Wang S, Ma A, Song S, Quan Q, Zhao X, Zheng X: Fasting serum free fatty acid composition, waist/hip ratio and insulin activity in essential hypertensive patients. Hypertens Res. 2008 Apr;31(4):623-32. [PubMed ]
HMDB01999	Eicosapentaenoic acid	Blood	Wang S, Ma A, Song S, Quan Q, Zhao X, Zheng X: Fasting serum free fatty acid composition, waist/hip ratio and insulin activity in essential hypertensive patients. Hypertens Res. 2008 Apr;31(4):623-32. [PubMed ]
HMDB00673	Linoleic acid	Blood; CSF; Urine	Wang S, Ma A, Song S, Quan Q, Zhao X, Zheng X: Fasting serum free fatty acid composition, waist/hip ratio and insulin activity in essential hypertensive patients. Hypertens Res. 2008 Apr;31(4):623-32. [PubMed ]

Essential Hypertension (EH) (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB01539	Dimethyl-L-arginine	Blood; CSF; Urine	Surdacki A, Nowicki M, Sandmann J, Tsikas D, Boeger RH, Bode-Boeger SM, Kruszelnicka-Kwiatkowska O, Kokot F, Dubiel JS, Froelich JC: Reduced urinary excretion of nitric oxide metabolites and increased plasma levels of asymmetric dimethylarginine in men with essential hypertension. J Cardiovasc Pharmacol. 1999 Apr;33(4):652-8. [PubMed ]

Ethylene Glycol Poisoning (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00115	Glycolic acid	Blood; CSF; Urine	Jacobsen D, Hewlett TP, Webb R, Brown ST, Ordinario AT, McMartin KE: Ethylene glycol intoxication: evaluation of kinetics and crystalluria. Am J Med. 1988 Jan;84(1):145-52. [PubMed ]

Ethylmalonic encephalopathy (5 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00339	2-Methylbutyrylglycine	Urine	Metagene: ETHYLMALONIC ENCEPHALOPATHY
HMDB00730	Isobutyrylglycine	Urine	Metagene: ETHYLMALONIC ENCEPHALOPATHY
HMDB00678	Isovalerylglycine	Blood; Urine	Metagene: ETHYLMALONIC ENCEPHALOPATHY
HMDB01844	Methylsuccinic acid	Urine	Metagene: ETHYLMALONIC ENCEPHALOPATHY
HMDB00808	N-Butyrylglycine	Urine	Metagene: ETHYLMALONIC ENCEPHALOPATHY

Eucalyptol exposure (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB04472	Eucalyptol	Blood	Jager W, Nasel B, Nasel C, Binder R, Stimpfl T, Vycudilik W, Buchbauer G: Pharmacokinetic studies of the fragrance compound 1,8-cineol in humans during inhalation. Chem Senses. 1996 Aug;21(4):477-80. [PubMed ]

Eugonadism (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00628	Epitestosterone	Blood	Kicman AT, Coutts SB, Cowan DA, Handelsman DJ, Howe CJ, Burring S, Wu FC: Adrenal and gonadal contributions to urinary excretion and plasma concentration of epitestosterone in men--effect of adrenal stimulation and implications for detection of testosterone abuse. Clin Endocrinol (Oxf). 1999 May;50(5):661-8. [PubMed ]

Euthyroidism (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB02728	Thyroxine sulfate	Blood	Chopra IJ, Santini F, Hurd RE, Chua Teco GN: A radioimmunoassay for measurement of thyroxine sulfate. J Clin Endocrinol Metab. 1993 Jan;76(1):145-50. [PubMed ]

Exercise (2 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00068	Epinephrine	Blood; CSF; Urine	Peyrin L, Pequignot JM, Lacour JR, Fourcade J: Relationships between catecholamine or 3-methoxy 4-hydroxy phenylglycol changes and the mental performance under submaximal exercise in man. Psychopharmacology (Berl). 1987;93(2):188-92. [PubMed ]
HMDB00216	Norepinephrine	Blood; CSF; Urine	Peyrin L, Pequignot JM, Lacour JR, Fourcade J: Relationships between catecholamine or 3-methoxy 4-hydroxy phenylglycol changes and the mental performance under submaximal exercise in man. Psychopharmacology (Berl). 1987;93(2):188-92. [PubMed ]

Extrahepatic biliary atresia (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00138	Glycocholic acid	Blood; Urine	Matsui A, Psacharopoulos HT, Mowat AP, Portmann B, Murphy GM: Radioimmunoassay of serum glycocholic acid, standard laboratory tests of liver function and liver biopsy findings: comparative study of children with liver disease. J Clin Pathol. 1982 Sep;35(9):1011-7. [PubMed ]

Extrahepatic biliary atresia (EHBA) (3 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00518	Chenodeoxycholic acid	Bile; Blood; Urine	Gustafsson J, Alvelius G, Bjorkhem I, Nemeth A: Bile acid metabolism in extrahepatic biliary atresia: lithocholic acid in stored dried blood collected at neonatal screening. Ups J Med Sci. 2006;111(1):131-6. [PubMed ]
HMDB00619	Cholic acid	Bile; Blood; Urine	Gustafsson J, Alvelius G, Bjorkhem I, Nemeth A: Bile acid metabolism in extrahepatic biliary atresia: lithocholic acid in stored dried blood collected at neonatal screening. Ups J Med Sci. 2006;111(1):131-6. [PubMed ]
HMDB00761	Lithocholic acid	Bile; Blood; Urine	Gustafsson J, Alvelius G, Bjorkhem I, Nemeth A: Bile acid metabolism in extrahepatic biliary atresia: lithocholic acid in stored dried blood collected at neonatal screening. Ups J Med Sci. 2006;111(1):131-6. [PubMed ]

Fabry disease (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB04948	Ceramide (d18:1/9Z-18:1)	Blood	Groener JE, Poorthuis BJ, Kuiper S, Helmond MT, Hollak CE, Aerts JM: HPLC for simultaneous quantification of total ceramide, glucosylceramide, and ceramide trihexoside concentrations in plasma. Clin Chem. 2007 Apr;53(4):742-7. Epub 2007 Mar 1. [PubMed ]

Familial Mediterranean Fever (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00490	Etiocholanolone	Blood; Urine	George JM, Wolff SM, Diller E, Bartter FC: Recurrent fever of unknown etiology: failure to demonstrate association between fever and plasma unconjugated etiocholanolone. J Clin Invest. 1969 Mar;48(3):558-63. [PubMed ]

Fatally injured drivers (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB01927	Diphenhydramine	Blood	Schwilke EW, Sampaio dos Santos MI, Logan BK: Changing patterns of drug and alcohol use in fatally injured drivers in Washington State. J Forensic Sci. 2006 Sep;51(5):1191-8. [PubMed ]

Fatty Acid Oxidation disorder with supplementation of MCT formula (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00387	3-Hydroxydodecanoic acid	Blood	Jones PM, Quinn R, Fennessey PV, Tjoa S, Goodman SI, Fiore S, Burlina AB, Rinaldo P, Boriack RL, Bennett MJ: Improved stable isotope dilution-gas chromatography-mass spectrometry method for serum or plasma free 3-hydroxy-fatty acids and its utility for the study of disorders of mitochondrial fatty acid beta-oxidation. Clin Chem. 2000 Feb;46(2):149-55. [PubMed ]

Febrile convulsions (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00112	Gamma-Aminobutyric acid	Blood; CSF; Saliva; Urine	Rating D, Siemes H, Loscher W: Low CSF GABA concentration in children with febrile convulsions, untreated epilepsy, and meningitis. J Neurol. 1983;230(4):217-25. [PubMed ]

Folate Deficiency (2 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00121	Folic acid	Blood; Cellular Cytoplasm; CSF; Urine	Wu AHB. Tietz clinical guide to laboratory tests. 4th ed. St. Louis: Saunders/Elsevier 2006.
HMDB00099	L-Cystathionine	Blood; CSF; Urine	Stabler SP, Lindenbaum J, Savage DG, Allen RH: Elevation of serum cystathionine levels in patients with cobalamin and folate deficiency. Blood. 1993 Jun 15;81(12):3404-13. [PubMed ]

Friedreich's ataxia (5 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00763	5-Hydroxyindoleacetic acid	Blood; CSF; Urine	Botez MI, Young SN: Biogenic amine metabolites and thiamine in cerebrospinal fluid in heredo-degenerative ataxias. Can J Neurol Sci. 2001 May;28(2):134-40. [PubMed ]
HMDB00118	Homovanillic acid	Blood; CSF; Urine	Botez MI, Young SN: Biogenic amine metabolites and thiamine in cerebrospinal fluid in heredo-degenerative ataxias. Can J Neurol Sci. 2001 May;28(2):134-40. [PubMed ]
HMDB00929	L-Tryptophan	Blood; CSF; Saliva; Urine	Botez MI, Young SN: Biogenic amine metabolites and thiamine in cerebrospinal fluid in heredo-degenerative ataxias. Can J Neurol Sci. 2001 May;28(2):134-40. [PubMed ]
HMDB00235	Thiamine	Blood; CSF; Urine	Pedraza OL, Botez MI: Thiamine status in inherited degenerative ataxias. J Neurol Neurosurg Psychiatry. 1992 Feb;55(2):136-7. [PubMed ]
HMDB01490	Vanylglycol	Blood; CSF; Urine	Botez MI, Young SN: Biogenic amine metabolites and thiamine in cerebrospinal fluid in heredo-degenerative ataxias. Can J Neurol Sci. 2001 May;28(2):134-40. [PubMed ]

Fumaric Aciduria (3 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00051	Ammonia	Blood; Cellular Cytoplasm; CSF; Urine	Metagene: FUMARIC ACIDURIA
HMDB00134	Fumaric acid	Blood; CSF; Urine	Metagene: FUMARIC ACIDURIA
HMDB00208	Oxoglutaric acid	Blood; Cellular Cytoplasm; CSF; N/A; Urine	Metagene: FUMARIC ACIDURIA

Functional hypothalamic amenorrhea (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00063	Cortisol	Blood; CSF; Saliva; Urine	Brundu B, Loucks TL, Adler LJ, Cameron JL, Berga SL: Increased cortisol in the cerebrospinal fluid of women with functional hypothalamic amenorrhea. J Clin Endocrinol Metab. 2006 Apr;91(4):1561-5. Epub 2006 Feb 7. [PubMed ]

GABA transaminase deficiency (2 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00056	Beta-Alanine	Blood; CSF; Urine	Metagene: GABA TRANSAMINASE DEFICIENCY
HMDB00112	Gamma-Aminobutyric acid	Blood; CSF; Saliva; Urine	Metagene: GABA TRANSAMINASE DEFICIENCY

Galactose-1-phosphate uridyltransferase deficiency (galactosemia) (1 metabolite)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00143	D-Galactose	Blood; Cellular Cytoplasm; CSF; Urine	Ning C, Segal S: Plasma galactose and galactitol concentration in patients with galactose-1-phosphate uridyltransferase deficiency galactosemia: determination by gas chromatography/mass spectrometry. Metabolism. 2000 Nov;49(11):1460-6. [PubMed ]

Galactosemia (4 metabolites)

HMDB ID	Name	Biofluid Location(s)	References
HMDB00143	D-Galactose	Blood; Cellular Cytoplasm; CSF; Urine	Chen J, Yager C, Reynolds R, Palmieri M, Segal S: Erythrocyte galactose 1-phosphate quantified by isotope-dilution gas chromatography-mass spectrometry. Clin Chem. 2002;48(4):604-12. [PubMed ]
HMDB00107	Galactitol	Amniotic Fluid; Blood; CSF; Urine	Jakobs C, Schweitzer S, Dorland B: Galactitol in galactosemia. Eur J Pediatr. 1995;154(7 Suppl 2):S50-2. [PubMed ]
HMDB00565	Galactonic acid	Blood; Urine	Yager CT, Chen J, Reynolds R, Segal S: Galactitol and galactonate in red blood cells of galactosemic patients. Mol Genet Metab. 2003 Nov;80(3):283-9. [

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Human Metabolome Database Version 2.5

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