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Human Metabolome Database Version 2.5

 

Showing metabocard for Uric acid (HMDB00289)

Legend: metabolite field enzyme field

Version 2.5
Creation Date 2005-11-16 15:48:42
Update Date 2011-06-21 10:16:49
Accession Number HMDB00289
Secondary Accession Numbers Not Available
Common Name Uric acid
Description Uric acid is a heterocyclic purine derivative that is the final oxidation product of purine metabolism. It is produced by the enzyme xanthine oxidase, which oxidizes oxypurines such as xanthine into uric acid. In most mammals, except humans and higher primates, the enzyme uricase further oxidizes uric acid to allantoin. Uric acid is also the end product of nitrogen metabolism in birds and reptiles. In such species, it is excreted in feces as a dry mass. Humans produce only small quantities of uric acid with excess accumulation leading to a type of arthritis known as gout. The loss of uricase in higher primates parallels the similar loss of the ability to synthesize ascorbic acid vitamin C. This may be because in higher primates uric acid partially replaces ascorbic acid.
Synonyms
  1. 1H-Purine-2,6,8-triol
  2. 2,6,8-Trihydroxypurine
  3. 2,6,8-Trioxopurine
  4. 2,6,8-Trioxypurine
  5. Lithate
  6. Lithic acid
  7. Purine-2,6,8(1H,3H,9H)-trione
  8. Urate
  9. Uric acid
Chemical IUPAC Name 7,9-dihydro-1H-Purine-2,6,8(3H)-trione
Chemical Formula C5H4N4O3
Chemical Structure Structure
Chemical Taxonomy
Kingdom
  • Organic
Super Class
  • Nucleosides and Nucleoside conjugates
Class
  • Purines and Purine Derivatives
Sub Class
  • Hydroxy purines
Family
  • Mammalian Metabolite
Species
  • oxo(het)arene
  • aromatic compound
  • heterocyclic compound
Biofunction
  • Electron donor
Application
—
Source
  • Endogenous
Average Molecular Weight 168.110
Monoisotopic Molecular Weight 168.028336
Isomeric SMILES O=C1NC(=O)C2=C(N1)NC(=O)N2
Canonical SMILES O=C1NC(=O)C2=C(N1)NC(=O)N2
KEGG Compound ID C00366 Link Image
BioCyc ID URATE Link Image
BiGG ID 34768 Link Image
Wikipedia Link Uric acid Link Image
NuGOwiki Link HMDB00289 Link Image
Metagene Link HMDB00289 Link Image
METLIN ID 88 Link Image
PubChem Compound 1175 Link Image
PubChem Substance 5428230 Link Image
ChEBI ID 17775 Link Image
CAS Registry Number 69-93-2
InChI Identifier InChI=1/C5H4N4O3/c10-3-1-2(7-4(11)6-1)8-5(12)9-3/h(H4,6,7,8,9,10,11,12)
Synthesis Reference Brenner-Holzach, O.; Leuthardt, F. Uric acid formation from glucose carbon in Drosophila melanogaster. Preliminary report. Helvetica Chimica Acta (1963), 46(4), 1426-8.
Melting Point (Experimental) > 300 oC
Experimental Water Solubility 0.06 mg/mL [YALKOWSKY,SH & DANNENFELSER,RM (1992)] Source: PhysProp
Predicted Water Solubility 1.76 mg/mL [Predicted by ALOGPS] Calculated using ALOGPS
Physiological Charge 0
State Solid
Experimental LogP/Hydrophobicity -2.17 [NAHUM,A & HORVATH,C (1980)] Source: PhysProp
Predicted LogP/Hydrophobicity -1.12 [Predicted by ALOGPS]; -1.6 [Predicted by PubChem via XLOGP] Calculated using ALOGPS
Material Safety Data Sheet (MSDS)
MOL File Show Link Image
SDF File Show Link Image
PDB File Show Link Image
2D Structure
3D Structure
Experimental PDB ID 1L5S Link Image
Experimental PDB File Show
Experimental PDB Structure
Experimental 1H NMR Spectrum
Show Experimental Conditions Link Image
Experimental 13C NMR Spectrum Not Available
Experimental 13C HSQC Spectrum
Show Experimental Conditions Link Image
Predicted 1H NMR Spectrum Show Image
Show Peaklist
Predicted 13C NMR Spectrum Show Image
Show Peaklist
Mass Spectrum
Low Energy
Download File
Show Experimental Conditions Link Image
Medium Energy
Download File
Show Experimental Conditions Link Image
High Energy
Download File
Show Experimental Conditions Link Image
Simplified TOCSY Spectrum Not Available
BMRB Spectrum Not Available
Cellular Location
  • Cytoplasm (Predicted from LogP)
  • Extracellular
  • peroxisome
Biofluid Location
  • Amniotic Fluid
  • Bile
  • Blood
  • Cerebrospinal Fluid
  • Saliva
  • Urine
Tissue Location
Tissue References
Adipose Tissue —
Bladder —
Brain —
Epidermis —
Erythrocyte —
Intestine —
Kidney —
Liver —
Muscle —
Placenta —
Platelet —
Spleen —
Concentrations (Normal)
Biofluid Amniotic Fluid
Value 384.18 +/- 203.31 uM
Age Adult:>18 yrs old
Sex Both
Patient information Normal
Comments Not Available
References
  • 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 Link Image]
Biofluid Bile
Value 260.0 +/- 63.0 uM
Age Adult:>18 yrs old
Sex Female
Patient information Normal glucose tolerance
Comments Not Available
References
  • 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 Link Image]
Biofluid Blood
Value 377.6 +/- 82.6 uM
Age Adult:>18 yrs old
Sex Male
Patient information Normal glucose tolerance
Comments Normal glucose tolerance according to the World Health Organization (WHO) criteria.
References
  • 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 Link Image]
Biofluid Blood
Value 372.0 (238.0-506.0) uM
Age Adult:>18 yrs old
Sex Male
Patient information Normal
Comments Not Available
References
  • The Merck Manual, 17th ed. Mark H. Beers, MD, Robert Berkow, MD, eds. Whitehouse Station, NJ: Merck Research Labs, 1999.
Biofluid Blood
Value 298.0 (149.0-446.0) uM
Age Adult:>18 yrs old
Sex Female
Patient information Normal
Comments Not Available
References
  • The Merck Manual, 17th ed. Mark H. Beers, MD, Robert Berkow, MD, eds. Whitehouse Station, NJ: Merck Research Labs, 1999.
Biofluid Blood
Value 329.0 +/- 129.0 uM
Age Newborn:0-30 days old
Sex Both
Patient information Normal
Comments Not Available
References
  • 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.
Biofluid Blood
Value 302.0 +/- 60.0 uM
Age Adult:>18 yrs old
Sex Male
Patient information Normal
Comments Not Available
References
  • 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.
Biofluid Blood
Value 234.0 +/- 52.0 uM
Age Adult:>18 yrs old
Sex Female
Patient information Normal
Comments Not Available
References
  • 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.
Biofluid Blood
Value 256.8 +/- 7.0 uM
Age Adult:>18 yrs old
Sex Both
Patient information Normal
Comments Not Available
References
  • 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 Link Image]
Biofluid Blood
Value 271.95 +/- 43.13 uM
Age Adult:>18 yrs old
Sex Both
Patient information Normal
Comments Not Available
References
  • 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 Link Image]
Biofluid CSF
Value 16.0 (4.2-28.0) uM
Age Adult:>18 yrs old
Sex Female
Patient information Normal
Comments Not Available
References
  • 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.
Biofluid CSF
Value 22.0 (6.6-37.0) uM
Age Adult:>18 yrs old
Sex Male
Patient information Normal
Comments Not Available
References
  • 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.
Biofluid CSF
Value 29.3 (11.9-46.7) uM
Age Adult:>18 yrs old
Sex Both
Patient information Normal
Comments Not Available
References
  • Kastenbauer S, Koedel U, Becker BF, Pfister HW: Oxidative stress in bacterial meningitis in humans. Neurology. 2002 Jan 22;58(2):186-91. [PubMed Link Image]
Biofluid CSF
Value 15.4 uM
Age Adult:>18 yrs old
Sex Both
Patient information Normal
Comments Not Available
References
  • GC/MS analysis by Josh Morrissey
Biofluid Saliva
Value <1.00 uM
Age Adult:>18 yrs old
Sex Both
Patient information Normal
Comments Not Available
References
  • Inoue K, Namiki T, Iwasaki Y, Yoshimura Y, Nakazawa H: Determination of uric acid in human saliva by high-performance liquid chromatography with amperometric electrochemical detection. J Chromatogr B Analyt Technol Biomed Life Sci. 2003 Feb 25;785(1):57-63. [PubMed Link Image]
Biofluid Urine
Value 118.09 +/- 114.57 umol/mmol creatinine
Age Infant:0-1 yr old
Sex Both
Patient information Normal
Comments Not Available
References
  • Shoemaker JD, Elliott WH: Automated screening of urine samples for carbohydrates, organic and amino acids after treatment with urease. J Chromatogr. 1991 Jan 2;562(1-2):125-38. [PubMed Link Image]
Biofluid Urine
Value 188.0 (78.9-296.0) umol/mmol creatinine
Age Adult:>18 yrs old
Sex Both
Patient information Normal
Comments Not Available
References
  • Merck Manual
Biofluid Urine
Value 524.75 +/- 249.57 umol/mmol creatinine
Age Children:1-13 yrs old
Sex Both
Patient information Normal
Comments Not Available
References
  • 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 Link Image]
Biofluid Urine
Value 337.84 +/- 29.75 umol/mmol creatinine
Age Adolescent:13-18 yrs old
Sex Both
Patient information Normal
Comments Not Available
References
  • 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 Link Image]
Biofluid Urine
Value 209.55 +/- 104.52 umol/mmol creatinine
Age Adult:>18 yrs old
Sex Both
Patient information Normal
Comments Not Available
References
  • 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 Link Image]
Biofluid Urine
Value 206.5 +/- 87.5 umol/mmol creatinine
Age Adult:>18 yrs old
Sex Both
Patient information Normal
Comments Not Available
References
  • 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.
Biofluid Urine
Value 166.45 +/- 31.5 umol/mmol creatinine
Age Adult:>18 yrs old
Sex Both
Patient information Normal
Comments Not Available
References
  • 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.
Biofluid Urine
Value 197.00 (98.6-293.5) umol/mmol creatinine
Age Adult:>18 yrs old
Sex Both
Patient information Normal
Comments Not Available
References
  • Kim KM, Henderson GN, Frye RF, Galloway CD, Brown NJ, Segal MS, Imaram W, Angerhofer A, Johnson RJ: Simultaneous determination of uric acid metabolites allantoin, 6-aminouracil, and triuret in human urine using liquid chromatography-mass spectrometry. J Chromatogr B Analyt Technol Biomed Life Sci. 2009 Jan 1;877(1-2):65-70. Epub 2008 Nov 25. [PubMed Link Image]
Concentrations (Abnormal)
Biofluid Blood
Value 400.0 +/- 103.2 uM
Age Adult:>18 yrs old
Sex Male
Condition Diabetes mellitus type 2
Comments High risk for type 2 diabetes. A higher risk of type 2 diabetes mellitus (DM) was defined as: (1) first-degree relative with DM or (2) previous fasting plasma glucose >= 5.4 mmol/L. Subjects were consecutively included from the outpatient clinic of the Diabetes Unit of the Hospital Clinic, who attended a diabetes prevention program. None had known their oral glucose tolerance state before inclusion in this study, and none was receiving a diuretic treatment.
References
  • 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 Link Image]
Biofluid Blood
Value 368.2 +/- 115.0 uM
Age Adult:>18 yrs old
Sex Female
Condition Diabetes mellitus type 2
Comments High risk for type 2 diabetes. A higher risk of type 2 diabetes mellitus (DM) was defined as: (1) first-degree relative with DM or (2) previous fasting plasma glucose >= 5.4 mmol/L. Subjects were consecutively included from the outpatient clinic of the Diabetes Unit of the Hospital Clinic, who attended a diabetes prevention program. None had known their oral glucose tolerance state before inclusion in this study, and none was receiving a diuretic treatment.
References
  • 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 Link Image]
Biofluid Blood
Value 426.0 +/- 107.4 uM
Age Adult:>18 yrs old
Sex Male
Condition Impaired glucose tolerance
Comments Impaired glucose tolerance according to the World Health Organization (WHO) criteria.
References
  • 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 Link Image]
Biofluid Blood
Value 347.5 +/- 105.0 uM
Age Adult:>18 yrs old
Sex Female
Condition Impaired glucose tolerance
Comments Impaired glucose tolerance according to the World Health Organization (WHO) criteria.
References
  • 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 Link Image]
Biofluid Blood
Value 1797.90 +/- 895.44 uM
Age Adult:>18 yrs old
Sex Both
Condition Canavan disease
Comments Not Available
References
  • 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 Link Image]
Biofluid Blood
Value 181.5 (35.7-327.0) uM
Age Adult:>18 yrs old
Sex Both
Condition Gout
Comments Not Available
References
Biofluid Blood
Value 476.00 (357.00-714.00) uM
Age Adult:>18 yrs old
Sex Both
Condition Gout
Comments Not Available
References
Biofluid Blood
Value 178.5 (35.7-327.00) uM
Age Adult:>18 yrs old
Sex Both
Condition Lesch-Nyhan syndrome
Comments Not Available
References
Biofluid Blood
Value 654.6 (595.00-714.00) uM
Age Adult:>18 yrs old
Sex Both
Condition Lesch-Nyhan syndrome
Comments Not Available
References
Biofluid Blood
Value 600-1000 uM
Age Children:1-13 yrs old
Sex Both
Condition Lesch-Nyhan syndrome
Comments Not Available
References
  • Nyhan WL: Lesch-Nyhan Disease. J Hist Neurosci. 2005 Mar;14(1):1-10. [PubMed Link Image]
Biofluid Blood
Value 193.89 +/- 49.05 uM
Age Adult:>18 yrs old
Sex Both
Condition Multiple sclerosis
Comments Not Available
References
  • Toncev G, Milicic B, Toncev S, Samardzic G: Serum uric acid levels in multiple sclerosis patients correlate with activity of disease and blood-brain barrier dysfunction. Eur J Neurol. 2002 May;9(3):221-6. [PubMed Link Image]
Biofluid Blood
Value 178.9 +/- 107.0 uM
Age Adult:>18 yrs old
Sex Both
Condition Bacterial meningitis
Comments Not Available
References
  • Peng F, Zhang B, Zhong X, Li J, Xu G, Hu X, Qiu W, Pei Z: Serum uric acid levels of patients with multiple sclerosis and other neurological diseases. Mult Scler. 2008 Mar;14(2):188-96. Epub 2007 Oct 17. [PubMed Link Image]
Biofluid Blood
Value 612 +/- 36 uM
Age Adult:>18 yrs old
Sex Both
Condition Cachexia
Comments Not Available
References
  • Doehner W, Rauchhaus M, Florea VG, Sharma R, Bolger AP, Davos CH, Coats AJ, Anker SD: Uric acid in cachectic and noncachectic patients with chronic heart failure: relationship to leg vascular resistance. Am Heart J. 2001 May;141(5):792-9. [PubMed Link Image]
Biofluid CSF
Value 24.0 (20.1-27.9) uM
Age Adult:>18 yrs old
Sex Both
Condition Degenerative disc disease
Comments Not Available
References
  • 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 Link Image]
Biofluid CSF
Value 125.0 (35.5-215.0) uM
Age Adult:>18 yrs old
Sex Both
Condition Meningitis
Comments Bacterial
References
  • Kastenbauer S, Koedel U, Becker BF, Pfister HW: Oxidative stress in bacterial meningitis in humans. Neurology. 2002 Jan 22;58(2):186-91. [PubMed Link Image]
Biofluid CSF
Value 22.0 uM
Age Adult:>18 yrs old
Sex Both
Condition Adenylosuccinate lyase deficiency
Comments Not Available
References
  • 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 Link Image]
Biofluid Urine
Value 1554.14 +/- 748.85 umol/mmol creatinine
Age Adult:>18 yrs old
Sex Both
Condition Canavan disease
Comments Not Available
References
  • 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 Link Image]
Biofluid Urine
Value 1244.00 (565.5-1923.00) umol/mmol creatinine
Age Adult:>18 yrs old
Sex Both
Condition Sulfite oxidase deficiency
Comments Not Available
References
Biofluid Urine
Value 248.8 umol/mmol creatinine
Age Adult:>18 yrs old
Sex Both
Condition Sulfite oxidase deficiency
Comments Not Available
References
Associated Disorders
Condition References
Adenylosuccinate lyase deficiency
  • 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 Link Image]
Bacterial meningitis
  • Peng F, Zhang B, Zhong X, Li J, Xu G, Hu X, Qiu W, Pei Z: Serum uric acid levels of patients with multiple sclerosis and other neurological diseases. Mult Scler. 2008 Mar;14(2):188-96. Epub 2007 Oct 17. [PubMed Link Image]
Cachexia
  • Doehner W, Rauchhaus M, Florea VG, Sharma R, Bolger AP, Davos CH, Coats AJ, Anker SD: Uric acid in cachectic and noncachectic patients with chronic heart failure: relationship to leg vascular resistance. Am Heart J. 2001 May;141(5):792-9. [PubMed Link Image]
Canavan disease
  • 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 Link Image]
Degenerative disc disease
  • 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 Link Image]
Diabetes mellitus type 2
  • 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 Link Image]
Gout
Impaired glucose tolerance
  • 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 Link Image]
Lesch-Nyhan syndrome
Meningitis
  • Kastenbauer S, Koedel U, Becker BF, Pfister HW: Oxidative stress in bacterial meningitis in humans. Neurology. 2002 Jan 22;58(2):186-91. [PubMed Link Image]
Multiple sclerosis
  • Toncev G, Milicic B, Toncev S, Samardzic G: Serum uric acid levels in multiple sclerosis patients correlate with activity of disease and blood-brain barrier dysfunction. Eur J Neurol. 2002 May;9(3):221-6. [PubMed Link Image]
Sulfite oxidase deficiency
OMIM ID
Pathways
Name SMPDB Link KEGG Link
Purine Metabolism SMP00050 Link Image map00230 Link Image
General References
  1. Kanbay M, Akcay A, Huddam B, Usluogullari CA, Arat Z, Ozdemir FN, Haberal M: Influence of cyclosporine and tacrolimus on serum uric acid levels in stable kidney transplant recipients. Transplant Proc. 2005 Sep;37(7):3119-20. [PubMed Link Image]
  2. 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 Link Image]
  3. Hanvivadhanakul P, Akkasilpa S, Deesomchok U: Efficacy of benzbromarone compared to allopurinol in lowering serum uric acid level in hyperuricemic patients. J Med Assoc Thai. 2002 Jun;85 Suppl 1:S40-7. [PubMed Link Image]
  4. Kirschbaum B: Correlation studies of plasma paraoxonase activity and uric acid concentration with AAPH-Induced erythrocyte hemolysis in hemodialysis patients. Artif Organs. 2004 Mar;28(3):259-64. [PubMed Link Image]
  5. Sysyn GD, Rozycki HJ: Lack of prognostic significance of early elevated serum uric acid levels in low birthweight infants. Biol Neonate. 2003;83(4):253-7. [PubMed Link Image]
  6. Kastenbauer S, Koedel U, Becker BF, Pfister HW: Oxidative stress in bacterial meningitis in humans. Neurology. 2002 Jan 22;58(2):186-91. [PubMed Link Image]
  7. Tumgor G, Arikan C, Kilic M, Aydogdu S: Frequency of hyperuricemia and effect of calcineurin inhibitors on serum uric acid levels in liver transplanted children. Pediatr Transplant. 2006 Sep;10(6):665-8. [PubMed Link Image]
  8. Srinivasan S, Kalaiselvi P, Sakthivel R, Pragasam V, Muthu V, Varalakshmi P: Uric acid: an abettor or protector in calcium oxalate urolithiasis? Biochemical study in stone formers. Clin Chim Acta. 2005 Mar;353(1-2):45-51. [PubMed Link Image]
  9. Puig JG, Torres R, Ruilope LM: AT1 blockers and uric acid metabolism: are there relevant differences? J Hypertens Suppl. 2002 Jun;20(5):S29-31. [PubMed Link Image]
  10. Alderman M, Aiyer KJ: Uric acid: role in cardiovascular disease and effects of losartan. Curr Med Res Opin. 2004 Mar;20(3):369-79. [PubMed Link Image]
  11. Iwai N, Mino Y, Hosoyamada M, Tago N, Kokubo Y, Endou H: A high prevalence of renal hypouricemia caused by inactive SLC22A12 in Japanese. Kidney Int. 2004 Sep;66(3):935-44. [PubMed Link Image]
  12. Williams KP, Galerneau F: The role of serum uric acid as a prognostic indicator of the severity of maternal and fetal complications in hypertensive pregnancies. J Obstet Gynaecol Can. 2002 Aug;24(8):628-32. [PubMed Link Image]
  13. Inoue K, Namiki T, Iwasaki Y, Yoshimura Y, Nakazawa H: Determination of uric acid in human saliva by high-performance liquid chromatography with amperometric electrochemical detection. J Chromatogr B Analyt Technol Biomed Life Sci. 2003 Feb 25;785(1):57-63. [PubMed Link Image]
  14. 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 Link Image]
  15. Cacabelos R, Fernandez-Novoa L, Corzo L, Pichel V, Lombardi V, Kubota Y: Genomics and phenotypic profiles in dementia: implications for pharmacological treatment. Methods Find Exp Clin Pharmacol. 2004 Jul-Aug;26(6):421-44. [PubMed Link Image]
  16. Simkin PA, Hoover PL, Paxson CS, Wilson WF: Uric acid excretion: quantitative assessment from spot, midmorning serum and urine samples. Ann Intern Med. 1979 Jul;91(1):44-7. [PubMed Link Image]
  17. Mazzali M: Uric acid and transplantation. Semin Nephrol. 2005 Jan;25(1):50-5. [PubMed Link Image]
  18. Wikipedia Link Image
Metabolic Enzymes
  1. Xanthine dehydrogenase/oxidase
Enzyme 1 [top]
Enzyme 1 ID 5413
Enzyme 1 Name Xanthine dehydrogenase/oxidase
Enzyme 1 Synonyms
  1. Xanthine dehydrogenase
  2. XD
  3. Xanthine oxidase
  4. XO
  5. Xanthine oxidoreductase
Enzyme 1 Gene Name XDH
Enzyme 1 Protein Sequence >Xanthine dehydrogenase/oxidase 
MTADKLVFFVNGRKVVEKNADPETTLLAYLRRKLGLSGTKLGCGEGGCGACTVMLSKYDR
LQNKIVHFSANACLAPICSLHHVAVTTVEGIGSTKTRLHPVQERIAKSHGSQCGFCTPGI
VMSMYTLLRNQPEPTMEEIENAFQGNLCRCTGYRPILQGFRTFARDGGCCGGDGNNPNCC
MNQKKDHSVSLSPSLFKPEEFTPLDPTQEPIFPPELLRLKDTPRKQLRFEGERVTWIQAS
TLKELLDLKAQHPDAKLVVGNTEIGIEMKFKNMLFPMIVCPAWIPELNSVEHGPDGISFG
AACPLSIVEKTLVDAVAKLPAQKTEVFRGVLEQLRWFAGKQVKSVASVGGNIITASPISD
LNPVFMASGAKLTLVSRGTRRTVQMDHTFFPGYRKTLLSPEEILLSIEIPYSREGEYFSA
FKQASRREDDIAKVTSGMRVLFKPGTTEVQELALCYGGMANRTISALKTTQRQLSKLWKE
ELLQDVCAGLAEELHLPPDAPGGMVDFRCTLTLSFFFKFYLTVLQKLGQENLEDKCGKLD
PTFASATLLFQKDPPADVQLFQEVPKGQSEEDMVGRPLPHLAADMQASGEAVYCDDIPRY
ENELSLRLVTSTRAHAKIKSIDTSEAKKVPGFVCFISADDVPGSNITGICNDETVFAKDK
VTCVGHIIGAVVADTPEHTQRAAQGVKITYEELPAIITIEDAIKNNSFYGPELKIEKGDL
KKGFSEADNVVSGEIYIGGQEHFYLETHCTIAVPKGEAGEMELFVSTQNTMKTQSFVAKM
LGVPANRIVVRVKRMGGGFGGKETRSTVVSTAVALAAYKTGRPVRCMLDRDEDMLITGGR
HPFLARYKVGFMKTGTVVALEVDHFSNVGNTQDLSQSIMERALFHMDNCYKIPNIRGTGR
LCKTNLPSNTAFRGFGGPQGMLIAECWMSEVAVTCGMPAEEVRRKNLYKEGDLTHFNQKL
EGFTLPRCWEECLASSQYHARKSEVDKFNKENCWKKRGLCIIPTKFGISFTVPFLNQAGA
LLHVYTDGSVLLTHGGTEMGQGLHTKMVQVASRALKIPTSKIYISETSTNTVPNTSPTAA
SVSADLNGQAVYAACQTILKRLEPYKKKNPSGSWEDWVTAAYMDTVSLSATGFYRTPNLG
YSFETNSGNPFHYFSYGVACSEVEIDCLTGDHKNLRTDIVMDVGSSLNPAIDIGQVEGAF
VQGLGLFTLEELHYSPEGSLHTRGPSTYKIPAFGSIPIEFRVSLLRDCPNKKAIYASKAV
GEPPLFLAASIFFAIKDAIRAARAQHTGNNVKELFRLDSPATPEKIRNACVDKFTTLCVT
GVPENCKPWSVRV
Enzyme 1 Number of Residues 1333
Enzyme 1 Molecular Weight 146423.0
Enzyme 1 Theoretical pI 7.70
Enzyme 1 GO Classification
Function
  • FAD or FADH2 binding
  • adenyl nucleotide binding
  • binding
  • catalytic activity
  • cation binding
  • electron carrier activity
  • ion binding
  • iron ion binding
  • iron-sulfur cluster binding
  • metal cluster binding
  • metal ion binding
  • molybdenum ion binding
  • nucleoside binding
  • oxidoreductase activity
  • oxidoreductase activity, acting on CH or CH2 groups
  • oxidoreductase activity, acting on CH or CH2 groups, NAD or NADP as acceptor
  • oxidoreductase activity, acting on CH or CH2 groups, oxygen as acceptor
  • purine nucleoside binding
  • transition metal ion binding
  • xanthine dehydrogenase activity
  • xanthine oxidase activity
Process
  • metabolic process
  • oxidation reduction
Component
—
Enzyme 1 General Function Involved in oxidoreductase activity
Enzyme 1 Specific Function Key enzyme in purine degradation. Catalyzes the oxidation of hypoxanthine to xanthine. Catalyzes the oxidation of xanthine to uric acid. Contributes to the generation of reactive oxygen species. Has also low oxidase activity towards aldehydes (in vitro)
Enzyme 1 Pathways
Enzyme 1 Reactions
  • xanthine + H2O + O2 = urate + H2O2 [RN:R02107]
Enzyme 1 Pfam Domain Function
Enzyme 1 Signals
  • None
Enzyme 1 Transmembrane Regions
  • None
Enzyme 1 Essentiality Not Available
Enzyme 1 GenBank ID Protein Not Available
Enzyme 1 UniProtKB/Swiss-Prot ID P47989 Link Image
Enzyme 1 UniProtKB/Swiss-Prot Entry Name XDH_HUMAN Link Image
Enzyme 1 PDB ID 1V97 Link Image
Enzyme 1 PDB File Show
Enzyme 1 3D Structure
Enzyme 1 Cellular Location Not Available
Enzyme 1 Gene Sequence >4002 bp 
ATGACAGCAGACAAATTGGTTTTCTTTGTGAATGGCAGAAAGGTGGTGGAGAAAAATGCA
GATCCAGAGACAACCCTTTTGGCCTACCTGAGAAGAAAGTTGGGGCTGAGTGGAACCAAG
CTCGGCTGTGGAGAGGGGGGCTGCGGGGCTTGCACAGTGATGCTCTCCAAGTATGATCGT
CTGCAGAACAAGATCGTCCACTTTTCTGCCAATGCCTGCCTGGCCCCCATCTGCTCCTTG
CACCATGTTGCAGTGACAACTGTGGAAGGAATAGGAAGCACCAAGACGAGGCTGCATCCT
GTGCAGGAGAGAATTGCCAAAAGCCACGGCTCCCAGTGCGGGTTCTGCACCCCTGGCATC
GTCATGAGTATGTACACACTGCTCCGGAATCAGCCCGAGCCCACCATGGAGGAGATTGAG
AATGCCTTCCAAGGAAATCTGTGCCGCTGCACAGGCTACAGACCCATCCTCCAGGGCTTC
CGGACCTTTGCCAGGGATGGTGGATGCTGTGGAGGAGATGGGAATAATCCAAATTGCTGC
ATGAACCAGAAGAAAGACCACTCAGTCAGCCTCTCGCCATCTTTATTCAAACCAGAGGAG
TTCACGCCCCTGGATCCAACCCAGGAGCCCATTTTTCCCCCAGAGTTGCTGAGGCTGAAA
GACACTCCTCGGAAGCAGCTGCGATTTGAAGGGGAGCGTGTGACGTGGATACAGGCCTCA
ACCCTCAAGGAGCTGCTGGACCTCAAGGCTCAGCACCCTGACGCCAAGCTGGTCGTGGGG
AACACGGAGATTGGCATTGAGATGAAGTTCAAGAATATGCTGTTTCCTATGATTGTCTGC
CCAGCCTGGATCCCTGAGCTGAATTCGGTAGAACATGGACCCGACGGTATCTCCTTTGGA
GCTGCTTGCCCCCTGAGCATTGTGGAAAAAACCCTGGTGGATGCTGTTGCTAAGCTTCCT
GCCCAAAAGACAGAGGTGTTCAGAGGGGTCCTGGAGCAGCTGCGCTGGTTTGCTGGGAAG
CAAGTCAAGTCTGTGGCGTCCGTTGGAGGGAACATCATCACTGCCAGCCCCATCTCCGAC
CTCAACCCCGTGTTCATGGCCAGTGGGGCCAAGCTGACACTTGTGTCCAGAGGCACCAGG
AGAACTGTCCAGATGGACCACACCTTCTTCCCTGGCTACAGAAAGACCCTGCTGAGCCCG
GAGGAGATACTGCTCTCCATAGAGATCCCCTACAGCAGGGAGGGGGAGTATTTCTCAGCA
TTCAAGCAGGCCTCCCGGAGAGAAGATGACATTGCCAAGGTAACCAGTGGCATGAGAGTT
TTATTCAAGCCAGGAACCACAGAGGTACAGGAGCTGGCCCTTTGCTATGGTGGAATGGCC
AACAGAACCATCTCAGCCCTCAAGACCACTCAGAGGCAGCTTTCCAAGCTCTGGAAGGAG
GAGCTGCTGCAGGACGTGTGTGCAGGACTGGCAGAGGAGCTGCATCTGCCTCCCGATGCC
CCTGGTGGCATGGTGGACTTCCGGTGCACCCTCACCCTCAGCTTCTTCTTCAAGTTCTAC
CTGACAGTCCTTCAGAAGCTGGGCCAAGAGAACCTGGAAGACAAGTGTGGTAAACTGGAC
CCCACTTTCGCCAGTGCAACTTTACTGTTTCAGAAAGACCCCCCAGCCGATGTCCAGCTC
TTCCAAGAGGTGCCCAAGGGTCAGTCTGAGGAGGACATGGTGGGCCGGCCCCTGCCCCAC
CTGGCAGCGGACATGCAGGCCTCTGGTGAGGCCGTGTACTGTGACGACATTCCTCGCTAC
GAGAATGAGCTGTCTCTCCGGCTGGTCACCAGCACCCGGGCCCACGCCAAGATCAAGTCC
ATAGATACATCAGAAGCTAAGAAGGTTCCAGGGTTTGTTTGTTTCATTTCCGCTGATGAT
GTTCCTGGGAGTAACATAACTGGAATTTGTAATGATGAGACAGTCTTTGCGAAGGATAAG
GTTACTTGTGTTGGGCATATCATTGGTGCTGTGGTTGCTGACACCCCGGAACACACACAG
AGAGCTGCCCAAGGGGTGAAAATCACCTATGAAGAACTACCAGCCATTATCACAATTGAG
GATGCTATAAAGAACAACTCCTTTTATGGACCTGAGCTGAAGATCGAGAAAGGGGACCTA
AAGAAGGGGTTTTCCGAAGCAGATAATGTTGTGTCAGGGGAGATATACATCGGTGGCCAA
GAGCACTTCTACCTGGAGACTCACTGCACCATTGCTGTTCCAAAAGGCGAGGCAGGGGAG
ATGGAGCTCTTTGTGTCTACACAGAACACCATGAAGACCCAGAGCTTTGTTGCAAAAATG
TTGGGGGTTCCAGCAAACCGGATTGTGGTTCGAGTGAAGAGAATGGGAGGAGGCTTTGGA
GGCAAGGAGACCCGGAGCACTGTGGTGTCCACGGCAGTGGCCCTGGCTGCATATAAGACC
GGCCGCCCTGTGCGATGCATGCTGGACCGTGATGAGGACATGCTGATAACTGGTGGCAGA
CATCCCTTCCTGGCCAGATACAAGGTTGGCTTCATGAAGACTGGGACAGTTGTGGCTCTT
GAGGTGGACCACTTCAGCAATGTGGGGAACACCCAGGATCTCTCTCAGAGTATTATGGAA
CGAGCTTTATTCCACATGGACAACTGCTATAAAATCCCCAACATCCGGGGCACTGGGCGG
CTGTGCAAAACCAACCTTCCCTCCAACACGGCCTTCCGGGGCTTTGGGGGGCCCCAGGGG
ATGCTCATTGCCGAGTGCTGGATGAGTGAAGTTGCAGTGACCTGTGGGATGCCTGCAGAG
GAGGTGCGGAGAAAAAACCTGTACAAAGAAGGGGACCTGACACACTTCAACCAGAAGCTT
GAGGGTTTCACCTTGCCCAGATGCTGGGAAGAATGCCTAGCAAGCTCTCAGTATCATGCT
CGGAAGAGTGAGGTTGACAAGTTCAACAAGGAGAATTGTTGGAAAAAGAGAGGATTGTGC
ATAATTCCCACCAAGTTTGGAATAAGCTTCACAGTTCCTTTTCTGAATCAGGCAGGAGCC
CTACTTCATGTGTACACAGATGGCTCTGTGCTGCTGACCCACGGGGGGACTGAGATGGGC
CAAGGCCTTCATACCAAAATGGTCCAGGTGGCCAGTAGAGCTCTGAAAATCCCCACCTCT
AAGATTTATATCAGCGAGACAAGCACTAACACTGTGCCCAACACCTCTCCCACGGCTGCC
TCTGTCAGCGCTGACCTCAATGGACAGGCCGTCTATGCGGCTTGTCAGACCATCTTGAAA
AGGCTGGAACCCTACAAGAAGAAGAATCCCAGTGGCTCCTGGGAAGACTGGGTCACAGCT
GCCTACATGGACACAGTGAGCTTGTCTGCCACTGGGTTTTATAGAACACCCAATCTGGGC
TACAGCTTTGAGACTAACTCAGGGAACCCCTTCCACTACTTCAGCTATGGGGTGGCTTGC
TCTGAAGTAGAAATCGACTGCCTAACAGGAGATCATAAGAACCTCCGCACAGATATTGTC
ATGGATGTTGGCTCCAGTCTAAACCCTGCCATTGATATTGGACAGGTGGAAGGGGCATTT
GTCCAGGGCCTTGGCCTCTTCACCCTAGAGGAGCTACACTATTCCCCCGAGGGGAGCCTG
CACACCCGTGGCCCTAGCACCTACAAGATCCCGGCATTTGGCAGCATCCCCATTGAGTTC
AGGGTGTCCCTGCTCCGCGACTGCCCCAACAAGAAGGCCATCTATGCATCGAAGGCTGTT
GGAGAGCCGCCCCTCTTCCTGGCTGCTTCTATCTTCTTTGCCATCAAAGATGCCATCCGT
GCAGCTCGAGCTCAGCACACAGGTAATAACGTGAAGGAACTCTTCCGGCTAGACAGCCCT
GCCACCCCGGAGAAGATCCGCAATGCCTGCGTGGACAAGTTCACCACCCTGTGTGTCACT
GGTGTCCCAGAAAACTGCAAACCCTGGTCTGTGAGGGTCTAA
Enzyme 1 GenBank Gene ID D11456 Link Image
Enzyme 1 GeneCard ID XDH Link Image
Enzyme 1 GenAtlas ID XDH Link Image
Enzyme 1 HGNC ID HGNC:12805 Link Image
Enzyme 1 Chromosome Location 2
Enzyme 1 Locus 2p23.1
Enzyme 1 SNPs SNPJam Report Link Image
Enzyme 1 General References
  1. Ichida K, Amaya Y, Noda K, Minoshima S, Hosoya T, Sakai O, Shimizu N, Nishino T: Cloning of the cDNA encoding human xanthine dehydrogenase (oxidase): structural analysis of the protein and chromosomal location of the gene. Gene. 1993 Nov 15;133(2):279-84. [PubMed Link Image]
  2. Xu P, Huecksteadt TP, Harrison R, Hoidal JR: Molecular cloning, tissue expression of human xanthine dehydrogenase. Biochem Biophys Res Commun. 1994 Mar 15;199(2):998-1004. [PubMed Link Image]
  3. Xu P, Huecksteadt TP, Harrison R, Hoidal JR: Molecular cloning, tissue expression of human xanthine dehydrogenase. Biochem Biophys Res Commun. 1995 Oct 4;215(1):429. [PubMed Link Image]
  4. Saksela M, Raivio KO: Cloning and expression in vitro of human xanthine dehydrogenase/oxidase. Biochem J. 1996 Apr 1;315 ( Pt 1):235-9. [PubMed Link Image]
  5. Olsen JV, Blagoev B, Gnad F, Macek B, Kumar C, Mortensen P, Mann M: Global, in vivo, and site-specific phosphorylation dynamics in signaling networks. Cell. 2006 Nov 3;127(3):635-48. [PubMed Link Image]
  6. Ichida K, Amaya Y, Kamatani N, Nishino T, Hosoya T, Sakai O: Identification of two mutations in human xanthine dehydrogenase gene responsible for classical type I xanthinuria. J Clin Invest. 1997 May 15;99(10):2391-7. [PubMed Link Image]
  7. Levartovsky D, Lagziel A, Sperling O, Liberman U, Yaron M, Hosoya T, Ichida K, Peretz H: XDH gene mutation is the underlying cause of classical xanthinuria: a second report. Kidney Int. 2000 Jun;57(6):2215-20. [PubMed Link Image]
  8. Picariello G, Ferranti P, Mamone G, Roepstorff P, Addeo F: Identification of N-linked glycoproteins in human milk by hydrophilic interaction liquid chromatography and mass spectrometry. Proteomics. 2008 Sep;8(18):3833-47. [PubMed Link Image]
  9. Yamaguchi Y, Matsumura T, Ichida K, Okamoto K, Nishino T: Human xanthine oxidase changes its substrate specificity to aldehyde oxidase type upon mutation of amino acid residues in the active site: roles of active site residues in binding and activation of purine substrate. J Biochem. 2007 Apr;141(4):513-24. Epub 2007 Feb 14. [PubMed Link Image]
  10. Sakamoto N, Yamamoto T, Moriwaki Y, Teranishi T, Toyoda M, Onishi Y, Kuroda S, Sakaguchi K, Fujisawa T, Maeda M, Hada T: Identification of a new point mutation in the human xanthine dehydrogenase gene responsible for a case of classical type I xanthinuria. Hum Genet. 2001 Apr;108(4):279-83. [PubMed Link Image]
  11. Gok F, Ichida K, Topaloglu R: Mutational analysis of the xanthine dehydrogenase gene in a Turkish family with autosomal recessive classical xanthinuria. Nephrol Dial Transplant. 2003 Nov;18(11):2278-83. [PubMed Link Image]
  12. Sjoblom T, Jones S, Wood LD, Parsons DW, Lin J, Barber TD, Mandelker D, Leary RJ, Ptak J, Silliman N, Szabo S, Buckhaults P, Farrell C, Meeh P, Markowitz SD, Willis J, Dawson D, Willson JK, Gazdar AF, Hartigan J, Wu L, Liu C, Parmigiani G, Park BH, Bachman KE, Papadopoulos N, Vogelstein B, Kinzler KW, Velculescu VE: The consensus coding sequences of human breast and colorectal cancers. Science. 2006 Oct 13;314(5797):268-74. Epub 2006 Sep 7. [PubMed Link Image]
Enzyme 1 Metabolite References Not Available