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Human Metabolome Database Version 3.5

Showing metabocard for Glycine (HMDB00123)

• Identification
• Taxonomy
• Ontology
• Physical properties
• Spectra
• Biological properties
• Concentrations
• Links
• References

enzymes (38) transporters (2)

• Blood
• Urine

Record Information
Version	3.5
Creation Date	2005-11-16 08:48:42 -0700
Update Date	2013-02-08 17:07:55 -0700
HMDB ID	HMDB00123
Secondary Accession Numbers	None
Metabolite Identification
Common Name	Glycine
Description	Glycine is a simple, nonessential amino acid, although experimental animals show reduced growth on low-glycine diets. The average adult ingests 3 to 5 grams of glycine daily. Glycine is involved in the body's production of DNA, phospholipids and collagen, and in release of energy. Glycine levels are effectively measured in plasma in both normal patients and those with inborn errors of glycine metabolism. (http://www.dcnutrition.com/AminoAcids/) Nonketotic hyperglycinaemia (OMIM 606899 ) is an autosomal recessive condition caused by deficient enzyme activity of the glycine cleavage enzyme system (EC 2.1.1.10). The glycine cleavage enzyme system comprises four proteins: P-, T-, H- and L-proteins (EC 1.4.4.2, EC 2.1.2.10 and EC 1.8.1.4 for P-, T- and L-proteins). Mutations have been described in the GLDC (OMIM 238300 ), AMT (OMIM 238310 ), and GCSH (OMIM 238330 ) genes encoding the P-, T-, and H-proteins respectively. The glycine cleavage system catalyses the oxidative conversion of glycine into carbon dioxide and ammonia, with the remaining one-carbon unit transferred to folate as methylenetetrahydrofolate. It is the main catabolic pathway for glycine and it also contributes to one-carbon metabolism. Patients with a deficiency of this enzyme system have increased glycine in plasma, urine and cerebrospinal fluid (CSF) with an increased CSF: plasma glycine ratio. (PMID 16151895 ).
Structure	Download: MOL | SDF | SMILES | InChI Display: 2D Structure | 3D Structure
Synonyms	2-Aminoacetate 2-Aminoacetic acid Aciport Amino-Acetate Amino-Acetic acid Aminoacetate Aminoacetic acid Aminoethanoate Aminoethanoic acid Glicoamin Glycocoll Glycolixir Glycosthene Gyn-Hydralin Padil
Chemical Formula	C2H5NO2
Average Molecular Weight	75.0666
Monoisotopic Molecular Weight	75.032028409
IUPAC Name	2-aminoacetic acid
Traditional IUPAC Name	glycine
CAS Registry Number	56-40-6
SMILES	NCC(O)=O
InChI Identifier	InChI=1S/C2H5NO2/c3-1-2(4)5/h1,3H2,(H,4,5)
InChI Key	DHMQDGOQFOQNFH-UHFFFAOYSA-N
Chemical Taxonomy
Kingdom	Organic Compounds
Super Class	Amino Acids, Peptides, and Analogues
Class	Amino Acids and Derivatives
Sub Class	Alpha Amino Acids and Derivatives
Other Descriptors	Aliphatic Acyclic Compounds Amino acids(KEGG) Common amino acids(KEGG) amino acid zwitterion(ChEBI)
Substituents	Carboxylic Acid Primary Aliphatic Amine (Alkylamine)
Direct Parent	Alpha Amino Acids and Derivatives
Ontology
Status	Detected and Quantified
Origin	Endogenous
Biofunction	Non-essential amino acid
Application	Not Available
Cellular locations	Extracellular Mitochondria Lysosome Peroxisome
Physical Properties
State	Solid
Experimental Properties	Property Value Reference Melting Point 262.2 °C Not Available Boiling Point Not Available Not Available Water Solubility 249.0 mg/mL Not Available LogP -3.21 HANSCH,C ET AL. (1995)
Predicted Properties	Property Value Source Water Solubility 552 g/L ALOGPS LogP -3.34 ALOGPS LogP -3.4 ChemAxon LogS 0.87 ALOGPS pKa (strongest acidic) 2.31 ChemAxon pKa (strongest basic) 9.24 ChemAxon Hydrogen Acceptor Count 3 ChemAxon Hydrogen Donor Count 2 ChemAxon Polar Surface Area 63.32 A2 ChemAxon Rotatable Bond Count 1 ChemAxon Refractivity 16 ChemAxon Polarizability 6.65 ChemAxon Formal Charge 0 ChemAxon Physiological Charge 0 ChemAxon
Spectra
	Gas-MS Spectrum 1H NMR Spectrum 13C NMR Spectrum MS/MS Spectrum Quattro_QQQ 10 MS/MS Spectrum Quattro_QQQ 25 MS/MS Spectrum Quattro_QQQ 40 MS/MS Spectrum EI-B (HITACHI RMU-6M) MS/MS Spectrum LC-ESI-QQ (API3000, Applied Biosystems) 10 MS/MS Spectrum LC-ESI-QQ (API3000, Applied Biosystems) 20 MS/MS Spectrum LC-ESI-QQ (API3000, Applied Biosystems) 30 MS/MS Spectrum LC-ESI-QQ (API3000, Applied Biosystems) 10 MS/MS Spectrum LC-ESI-QQ (API3000, Applied Biosystems) 20 MS/MS Spectrum LC-ESI-QQ (API3000, Applied Biosystems) 30 MS/MS Spectrum LC-ESI-QQ (API3000, Applied Biosystems) 40 MS/MS Spectrum LC-ESI-QQ (API3000, Applied Biosystems) 50 MS/MS Spectrum GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) MS/MS Spectrum GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies ) MS/MS Spectrum GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) [1H,1H] 2D NMR Spectrum [1H,13C] 2D NMR Spectrum
Biological Properties
Cellular Locations	Extracellular Mitochondria Lysosome Peroxisome
Biofluid Locations	Bile Blood Cerebrospinal Fluid (CSF) Saliva Urine
Tissue Location	Bladder Fibroblasts Intestine Neuron Pancreas Placenta Kidney Epidermis Thyroid Gland Brain Myelin Prostate Platelet Spleen Stratum Corneum
Pathways	Name SMPDB Link KEGG Link Ammonia Recycling SMP00009 map00910 Bile Acid Biosynthesis SMP00035 map00120 Porphyrin Metabolism SMP00024 map00860 Glutathione Metabolism SMP00015 map00480 Glycine and Serine Metabolism SMP00004 map00260 Alanine Metabolism SMP00055 map00250 Methionine Metabolism SMP00033 map00270 Carnitine Synthesis SMP00465 Not Available
Normal Concentrations
	Biofluid Status Value Age Sex Condition Comments Bile Detected and Quantified >10 uM Adult (>18 years old) Both Normal Not Available Blood Detected and Quantified 242.0 +/- 44.0 uM Adult (>18 years old) Male Normal Not Available Blood Detected and Quantified 258.0 +/- 64.0 uM Adult (>18 years old) Female Normal Not Available Blood Detected and Quantified 329.9 +/- 105.6 uM Adult (>18 years old) Not Specified Normal Not Available Blood Detected and Quantified 280.0 (140.0-420.0) uM Newborn (0-30 days old) Both Normal Not Available Blood Detected and Quantified 212.4 +/- 57.4 uM Adult (>18 years old) Male Normal Not Available Blood Detected and Quantified 230.0 (178.0-282.0) uM Adult (>18 years old) Both Normal Not Available Blood Detected and Quantified 325.4 +/- 126.8 uM Adult (>18 years old) Not Specified Normal Not Available Blood Detected and Quantified 460.0 +/- 275.0 uM Newborn (0-30 days old) Not Specified Normal Not Available Blood Detected and Quantified 234.0 +/- 34.0 uM Children (1-13 year old) Male Normal Not Available Cerebrospinal Fluid (CSF) Detected and Quantified 4.7 +/- 1.5 uM Adult (>18 years old) Both Normal Not Available Cerebrospinal Fluid (CSF) Detected and Quantified 9.0 (3.0-19.0) uM Adult (>18 years old) Both Normal Not Available Cerebrospinal Fluid (CSF) Detected and Quantified 9.5 +/- 4.7 uM Adult (>18 years old) Not Specified Normal Not Available Cerebrospinal Fluid (CSF) Detected and Quantified 8.2 +/- 3.0 uM Adult (>18 years old) Not Specified Normal Not Available Cerebrospinal Fluid (CSF) Detected and Quantified 6.1 +/- 1.4 uM Adult (>18 years old) Not Specified Normal Not Available Cerebrospinal Fluid (CSF) Detected and Quantified 4.63 +/- 0.74 uM Adult (>18 years old) Both Normal Not Available Cerebrospinal Fluid (CSF) Detected and Quantified 8.30 (5.88-10.7) uM Adult (>18 years old) Both Normal Not Available Cerebrospinal Fluid (CSF) Detected and Quantified 11 +/- 4  uM Not Specified Not Specified Normal Not Available Saliva Detected and Quantified 1-472 uM Adult (>18 years old) Male normal At rest without stimulation Saliva Detected and Quantified 1-94 uM Adult (>18 years old) Male normal Salivary induction with a citric acid solution... Saliva Detected and Quantified >10 uM Adult (>18 years old) Both Normal Not Available Urine Detected and Quantified 75.7 +/- 51.7 umol/mmol creatinine Adult (>18 years old) Both Normal by GC-MS Urine Detected and not Quantified Not Applicable Adult (>18 years old) Male Normal Not Available Urine Detected and Quantified 165.0 umol/mmol creatinine Adult (>18 years old) Both Normal Not Available Urine Detected and Quantified 151(48-233) umol/mmol creatinine Adult (>18 years old) Both Normal Not Available Urine Detected and Quantified 119.95-599.74 umol/mmol creatinine Adult (>18 years old) Both Normal Not Available Urine Detected and Quantified 151.0 (233-248) umol/mmol creatinine Adult (>18 years old) Both Normal Not Available Urine Detected and not Quantified Not Applicable Adult (>18 years old) Both Normal Not Available Urine Detected and Quantified 513.54 +/- 360.61 umol/mmol creatinine Infant (0-1 year old) Both Normal Not Available Urine Detected and Quantified 106 (44-300) umol/mmol creatinine Adult (>18 years old) Both Normal urine by NMR Urine Detected and Quantified 0.0027 - 0.0682 umol/mmol creatinine Adult (>18 years old) Both Normal Not Available Urine Detected and Quantified 71.23 umol/mmol creatinine Adult (>18 years old) Male Normal Not Available Urine Detected and not Quantified Not Applicable Adult (>18 years old) Both Normal Urine compound detected by GC-MS Urine Detected and Quantified 128.1 +/- 65.5 umol/mmol creatinine Adult (>18 years old) Male Normal Not Available Urine Detected and Quantified 187.6 +/- 92.3 umol/mmol creatinine Adult (>18 years old) Female Normal Not Available Urine Detected and Quantified 15.0 (10.0-23.0) umol/mmol creatinine Newborn (0-30 days old) Both Normal Not Available Urine Detected and Quantified 30.0 +/- 19.0 umol/mmol creatinine Children (1-13 year old) Both Normal Not Available Urine Detected and not Quantified Not Applicable Adult (>18 years old) Male Normal After green tea sonsumption Urine Detected and Quantified 92.0 +/- 39.0 umol/mmol creatinine Adult (>18 years old) Male Normal Not Available Urine Detected and not Quantified Not Applicable Adult (>18 years old) Male Normal After black tea sonsumption Urine Detected and Quantified 139.0 +/- 86.0 umol/mmol creatinine Adult (>18 years old) Female Normal Not Available Urine Detected and Quantified 136.48 +/- 77.27 umol/mmol creatinine Adult (>18 years old) Both Normal Not Available Urine Detected and Quantified 101 (37.0-250.6) umol/mmol creatinine Adult (>18 years old) Both Normal by LC-MS/MS (Biocrates kit) Urine Detected and Quantified 118.9 umol/mmol creatinine Adult (>18 years old) Both Normal Not Available
Abnormal Concentrations
	Biofluid Status Value Age Sex Condition Comments Blood Detected and Quantified 234.9 +/- 181.1 uM Adult (>18 years old) Not Specified Heart Transplant Not Available Blood Detected and Quantified 1090.0 (780.0-1400.0) uM Newborn (0-30 days old) Both Non-ketotic hyperglycinemia Not Available Blood Detected and Quantified 486.0 (232.0-740.0) uM Adult (>18 years old) Both D-Glyceric aciduria Not Available Blood Detected and Quantified 1000.0 (800.0-1200.0) uM Adult (>18 years old) Both D-Glyceric acidura Not Available Blood Detected and Quantified 210.0 (203.0-217.0) uM Adult (>18 years old) Both Refractory localization-related epilepsy (RLE) Not Available Blood Detected and Quantified 354.0 (309.0-399.0) uM Children (1-13 year old) Both Juvenile myoclonic epilepsy (JME) Not Available Blood Detected and Quantified 0.153 +/- 0.012 uM Elderly (>65 years old) Both Alzheimer's disease Not Available Cerebrospinal Fluid (CSF) Detected and Quantified 9.7 +/- 4.9 uM Children (1-13 year old) Not Specified Leukemia Not Available Cerebrospinal Fluid (CSF) Detected and Quantified 13.4 +/- 6.4 uM Children (1-13 year old) Not Specified Leukemia Acute Lymphoblastic Leukemia (ALL) with Central... Cerebrospinal Fluid (CSF) Detected and Quantified 9.0405 (8.716-9.623) uM Not Specified Not Specified control Not Available Cerebrospinal Fluid (CSF) Detected and Quantified 4.5 +/- 0.75 uM Adult (>18 years old) Both Schizophrenia Not Available Cerebrospinal Fluid (CSF) Detected and Quantified 0.28 +/- 0.12 uM Adult (>18 years old) Both Alzheimer's disease Not Available Urine Detected and Quantified 3000.0 (1000.0-5000.0) umol/mmol creatinine Adult (>18 years old) Both Carbamoyl phosphate synthetase deficiency Not Available Urine Detected and Quantified 0.0024 - 0.0339 umol/mmol creatinine Adult (>18 years old) Both ADPKD Not Available Urine Detected and Quantified 84.0 +/- 67.0 umol/mmol creatinine Adult (>18 years old) Both Lung cancer Not Available Urine Detected and Quantified 0.010 +/- 0.001 umol/mmol creatinine Adult (>18 years old) Both Alzheimer's disease Not Available Urine Detected and Quantified 178.2 +/- 6.4 umol/mmol creatinine Adult (>18 years old) Both 3-Hydroxy-3- methylglutaryl-CoA lyase (HL) deficency Not Available Urine Detected and Quantified 135.8 +/- 5.8 umol/mmol creatinine Adult (>18 years old) Both 3-Hydroxy-3- methylglutaryl-CoA lyase (HL) deficency Not Available Urine Detected and Quantified 53.1 +/- 1.8 umol/mmol creatinine Adult (>18 years old) Both Arginosuccinic aciduria Not Available Urine Detected and Quantified 585.5 +/- 32.1 umol/mmol creatinine Adult (>18 years old) Both Propionic acidemia Not Available Urine Detected and Quantified 550.00 (100.00-1000.00) umol/mmol creatinine Adult (>18 years old) Both Iminoglycinuria Not Available Urine Detected and Quantified 2040.3 +/- 202.6 umol/mmol creatinine Adult (>18 years old) Both Propionic acidemia Not Available Urine Detected and Quantified 49.9 +/- 1.3 umol/mmol creatinine Adult (>18 years old) Both Tyrosinemia I Not Available Urine Detected and Quantified 176.2 +/- 9.2 umol/mmol creatinine Adult (>18 years old) Both Tyrosinemia I Not Available Urine Detected and Quantified 82.2 +/- 4.1 umol/mmol creatinine Adult (>18 years old) Both Phenylketonuria Not Available Urine Detected and Quantified 304.8 +/- 11.3 umol/mmol creatinine Adult (>18 years old) Both Phenylketonuria Not Available Urine Detected and Quantified 233.7 +/- 10.5 umol/mmol creatinine Adult (>18 years old) Both Maple syrup urine disease Not Available Urine Detected and Quantified 3205.1 +/- 83.4 umol/mmol creatinine Adult (>18 years old) Both Aminoaciduria Not Available
Associated Disorders and Diseases
Disease References	D-Glyceric acidemia Boneh A, Degani Y, Harari M: Prognostic clues and outcome of early treatment of nonketotic hyperglycinemia. Pediatr Neurol. 1996 Sep;15(2):137-41. Pubmed: 8888048 http://www.metagene.de/program/d.prg?mp=D-GLYCERIC%20ACIDURA Refractory localization-related epilepsy 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: 14992292 Juvenile myoclonic epilepsy 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: 14992292 Lung Cancer Wishart DS, Knox C, Guo AC, Eisner R, Young N, Gautam B, Hau DD, Psychogios N, Dong E, Bouatra S, Mandal R, Sinelnikov I, Xia J, Jia L, Cruz JA, Lim E, Sobsey CA, Shrivastava S, Huang P, Liu P, Fang L, Peng J, Fradette R, Cheng D, Tzur D, Clements M, Lewis A, De Souza A, Zuniga A, Dawe M, Xiong Y, Clive D, Greiner R, Nazyrova A, Shaykhutdinov R, Li L, Vogel HJ, Forsythe I: HMDB: a knowledgebase for the human metabolome. Nucleic Acids Res. 2008 Oct 25. Pubmed: 18953024 Leukemia Peng CT, Wu KH, Lan SJ, Tsai JJ, Tsai FJ, Tsai CH: Amino acid concentrations in cerebrospinal fluid in children with acute lymphoblastic leukemia undergoing chemotherapy. Eur J Cancer. 2005 May;41(8):1158-63. Epub 2005 Apr 14. Pubmed: 15911239 Alzheimer's disease 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: 17031479 Schizophrenia 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: 7595563 Carbamoyl Phosphate Synthetase Deficiency http://www.metagene.de/program/d.prg?mp=CARBAMOYL%20PHOSPHATE%20SYNTHETASE%20DEFICIENCY%20(CPS) Iminoglycinuria http://www.metagene.de/program/d.prg?mp=IMINOGLYCINURIA
Associated OMIM IDs	104300 (Alzheimer's disease) 237300 (Carbamoyl Phosphate Synthetase Deficiency) 605899 (D-Glyceric acidemia) 181500 (Schizophrenia) 606904 (Juvenile myoclonic epilepsy) 211980 (Lung Cancer) 242600 (Iminoglycinuria)
External Links
DrugBank ID	DB00145
Phenol Explorer Compound ID	Not Available
Phenol Explorer Metabolite ID	1062
FoodDB ID	FDB000484
KNApSAcK ID	C00001361
Chemspider ID	730
KEGG Compound ID	C00037
BioCyc ID	GLY
BiGG ID	33610
Wikipedia Link	Glycine
NuGOwiki Link	HMDB00123
Metagene Link	HMDB00123
METLIN ID	20
PubChem Compound	750
PDB ID	GLY
ChEBI ID	15428
References
Synthesis Reference	Anslow, Winston K.; King, Harold. Synthesis of glycine. Journal of the Chemical Society (1929), 2163-6.
Material Safety Data Sheet (MSDS)	Download (PDF)
General References	Christie GR, Ford D, Howard A, Clark MA, Hirst BH: Glycine supply to human enterocytes mediated by high-affinity basolateral GLYT1. Gastroenterology. 2001 Feb;120(2):439-48. Pubmed: 11159884 Jones CM, Smith M, Henderson MJ: Reference data for cerebrospinal fluid and the utility of amino acid measurement for the diagnosis of inborn errors of metabolism. Ann Clin Biochem. 2006 Jan;43(Pt 1):63-6. Pubmed: 16390611 Peng CT, Wu KH, Lan SJ, Tsai JJ, Tsai FJ, Tsai CH: Amino acid concentrations in cerebrospinal fluid in children with acute lymphoblastic leukemia undergoing chemotherapy. Eur J Cancer. 2005 May;41(8):1158-63. Epub 2005 Apr 14. Pubmed: 15911239 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: 12297216 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: 14992292 Bennett FI, Jackson AA: Glycine is not formed through the amino transferase reaction in human or rat placenta. Placenta. 1998 May;19(4):329-31. Pubmed: 9639330 Gomeza J, Ohno K, Hulsmann S, Armsen W, Eulenburg V, Richter DW, Laube B, Betz H: Deletion of the mouse glycine transporter 2 results in a hyperekplexia phenotype and postnatal lethality. Neuron. 2003 Nov 13;40(4):797-806. Pubmed: 14622583 Collins JW, Macdermott S, Bradbrook RA, Keeley FX Jr, Timoney AG: Is using ethanol-glycine irrigating fluid monitoring and 'good surgical practice' enough to prevent harmful absorption during transurethral resection of the prostate? BJU Int. 2006 Jun;97(6):1247-51. Pubmed: 16686720 Boneh A, Degani Y, Harari M: Prognostic clues and outcome of early treatment of nonketotic hyperglycinemia. Pediatr Neurol. 1996 Sep;15(2):137-41. Pubmed: 8888048 Dicke JM, Verges D, Kelley LK, Smith CH: Glycine uptake by microvillous and basal plasma membrane vesicles from term human placentae. Placenta. 1993 Jan-Feb;14(1):85-92. Pubmed: 8456092 Silwood CJ, Lynch E, Claxson AW, Grootveld MC: 1H and (13)C NMR spectroscopic analysis of human saliva. J Dent Res. 2002 Jun;81(6):422-7. Pubmed: 12097436 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: 2026685 Nicholson JK, O'Flynn MP, Sadler PJ, Macleod AF, Juul SM, Sonksen PH: Proton-nuclear-magnetic-resonance studies of serum, plasma and urine from fasting normal and diabetic subjects. Biochem J. 1984 Jan 15;217(2):365-75. Pubmed: 6696735 Prescot AP, de B Frederick B, Wang L, Brown J, Jensen JE, Kaufman MJ, Renshaw PF: In vivo detection of brain glycine with echo-time-averaged (1)H magnetic resonance spectroscopy at 4.0 T. Magn Reson Med. 2006 Mar;55(3):681-6. Pubmed: 16453318 Byard RW, Harrison R, Wells R, Gilbert JD: Glycine toxicity and unexpected intra-operative death. J Forensic Sci. 2001 Sep;46(5):1244-6. Pubmed: 11569574 Engelborghs S, Marescau B, De Deyn PP: Amino acids and biogenic amines in cerebrospinal fluid of patients with Parkinson's disease. Neurochem Res. 2003 Aug;28(8):1145-50. Pubmed: 12834252 Khan SA, Cox IJ, Hamilton G, Thomas HC, Taylor-Robinson SD: In vivo and in vitro nuclear magnetic resonance spectroscopy as a tool for investigating hepatobiliary disease: a review of H and P MRS applications. Liver Int. 2005 Apr;25(2):273-81. Pubmed: 15780050 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: 6321058 Hagenfeldt L, Bjerkenstedt L, Edman G, Sedvall G, Wiesel FA: Amino acids in plasma and CSF and monoamine metabolites in CSF: interrelationship in healthy subjects. J Neurochem. 1984 Mar;42(3):833-7. Pubmed: 6198473 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: 19212411

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Enzymes
Name: 5-aminolevulinate synthase, erythroid-specific, mitochondrial Reactions: succinyl-CoA + glycine = 5-aminolevulinate + CoA + CO2 [RN:R00830] Gene Name: ALAS2 Uniprot ID: P22557 Protein Sequence: FASTA Gene Sequence: FASTA
Name: 5-aminolevulinate synthase, nonspecific, mitochondrial Reactions: succinyl-CoA + glycine = 5-aminolevulinate + CoA + CO2 [RN:R00830] Gene Name: ALAS1 Uniprot ID: P13196 Protein Sequence: FASTA Gene Sequence: FASTA
Name: 2-amino-3-ketobutyrate coenzyme A ligase, mitochondrial Reactions: acetyl-CoA + glycine = CoA + L-2-amino-3-oxobutanoate [RN:R00371] Gene Name: GCAT Uniprot ID: O75600 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Peroxisomal sarcosine oxidase Reactions: L-pipecolate + O2 = 2,3,4,5-tetrahydropyridine-2-carboxylate + H2O2 [RN:R02204] Gene Name: PIPOX Uniprot ID: Q9P0Z9 Protein Sequence: FASTA Gene Sequence: FASTA
Name: D-amino-acid oxidase Reactions: a D-amino acid + H2O + O2 = a 2-oxo acid + NH3 + H2O2 [RN:R01340] Gene Name: DAO Uniprot ID: P14920 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Trifunctional purine biosynthetic protein adenosine-3 Reactions: ATP + 5-phospho-D-ribosylamine + glycine = ADP + phosphate + N1-(5-phospho-D-ribosyl)glycinamide [RN:R04144] Gene Name: GART Uniprot ID: P22102 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Alanine--glyoxylate aminotransferase 2, mitochondrial Reactions: L-alanine + glyoxylate = pyruvate + glycine [RN:R00369] Gene Name: AGXT2 Uniprot ID: Q9BYV1 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Glycine N-methyltransferase Reactions: S-adenosyl-L-methionine + glycine = S-adenosyl-L-homocysteine + sarcosine [RN:R00367] Gene Name: GNMT Uniprot ID: Q14749 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Aminopeptidase N Reactions: Release of an N-terminal amino acid, Xaa!Yaa- from a peptide, amide or arylamide. Xaa is preferably Ala, but may be most amino acids including Pro (slow action). When a terminal hydrophobic residue is followed by a prolyl residue, the two may be released as an intact Xaa-Pro dipeptide ALL_REAC (other) R00899 R04951 COFACTOR Manganese [CPD:C00034] Zinc [CPD:C00038] Gene Name: ANPEP Uniprot ID: P15144 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Glycine amidinotransferase, mitochondrial Reactions: L-arginine + glycine = L-ornithine + guanidinoacetate [RN:R00565] Gene Name: GATM Uniprot ID: P50440 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Bile acid-CoA:amino acid N-acyltransferase Reactions: palmitoyl-CoA + H2O = CoA + palmitate [RN:R01274] Gene Name: BAAT Uniprot ID: Q14032 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Glycyl-tRNA synthetase Reactions: ATP + glycine + tRNAGly = AMP + diphosphate + glycyl-tRNAGly [RN:R03654] Gene Name: GARS Uniprot ID: P41250 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Aminomethyltransferase, mitochondrial Reactions: [protein]-S8-aminomethyldihydrolipoyllysine + tetrahydrofolate = [protein]-dihydrolipoyllysine + 5,10-methylenetetrahydrofolate + NH3 [RN:R04125] Gene Name: AMT Uniprot ID: P48728 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Serine--pyruvate aminotransferase Reactions: L-serine + pyruvate = 3-hydroxypyruvate + L-alanine [RN:R00585] Gene Name: AGXT Uniprot ID: P21549 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Glutathione synthetase Reactions: ATP + gamma-L-glutamyl-L-cysteine + glycine = ADP + phosphate + glutathione [RN:R00497] Gene Name: GSS Uniprot ID: P48637 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Serine hydroxymethyltransferase, mitochondrial Reactions: 5,10-methylenetetrahydrofolate + glycine + H2O = tetrahydrofolate + L-serine [RN:R00945] Gene Name: SHMT2 Uniprot ID: P34897 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Sarcosine dehydrogenase, mitochondrial Reactions: sarcosine + acceptor + H2O = glycine + formaldehyde + reduced acceptor [RN:R00611] Gene Name: SARDH Uniprot ID: Q9UL12 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Serine hydroxymethyltransferase, cytosolic Reactions: 5,10-methylenetetrahydrofolate + glycine + H2O = tetrahydrofolate + L-serine [RN:R00945] Gene Name: SHMT1 Uniprot ID: P34896 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Glycine dehydrogenase [decarboxylating], mitochondrial Reactions: glycine + H-protein-lipoyllysine = H-protein-S-aminomethyldihydrolipoyllysine + CO2 [RN:R03425] Gene Name: GLDC Uniprot ID: P23378 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Cytosol aminopeptidase Reactions: Release of N-terminal proline from a peptide ALL_REAC (other) R00135 COFACTOR Manganese [CPD:C00034] Gene Name: LAP3 Uniprot ID: P28838 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Glutamate [NMDA] receptor subunit 3B Reactions: Gene Name: GRIN3B Uniprot ID: O60391 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Glutamate [NMDA] receptor subunit epsilon-1 Reactions: Gene Name: GRIN2A Uniprot ID: Q12879 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Glutamate [NMDA] receptor subunit epsilon-3 Reactions: Gene Name: GRIN2C Uniprot ID: Q14957 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Sodium- and chloride-dependent glycine transporter 1 Reactions: --- [] Gene Name: SLC6A9 Uniprot ID: P48067 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Glycine cleavage system H protein, mitochondrial Reactions: --- [] Gene Name: GCSH Uniprot ID: P23434 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Sodium- and chloride-dependent glycine transporter 2 Reactions: --- [] Gene Name: SLC6A5 Uniprot ID: Q9Y345 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Vesicular inhibitory amino acid transporter Reactions: Gene Name: SLC32A1 Uniprot ID: Q9H598 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Glycine N-acyltransferase Reactions: acyl-CoA + glycine = CoA + N-acylglycine [RN:R00395] Gene Name: GLYAT Uniprot ID: Q6IB77 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Glycine N-acyltransferase-like protein 1 Reactions: acyl-CoA + glycine = CoA + N-acylglycine [RN:R00395] Gene Name: GLYATL1 Uniprot ID: Q969I3 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Glycine N-acyltransferase-like protein 2 Reactions: acyl-CoA + glycine = CoA + N-acylglycine [RN:R00395] Gene Name: GLYATL2 Uniprot ID: Q8WU03 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Glycine receptor subunit alpha-1 Reactions: Gene Name: GLRA1 Uniprot ID: P23415 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Glycine receptor subunit alpha-2 Reactions: Gene Name: GLRA2 Uniprot ID: P23416 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Glycine receptor subunit alpha-3 Reactions: Gene Name: GLRA3 Uniprot ID: O75311 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Glycine receptor subunit beta Reactions: Gene Name: GLRB Uniprot ID: P48167 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Serine hydroxymethyltransferase Reactions: 5,10-methylenetetrahydrofolate + glycine + H2O = tetrahydrofolate + L-serine [RN:R00945] Gene Name: Not Available Uniprot ID: Q53ET4 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Serine hydroxymethyltransferase Reactions: 5,10-methylenetetrahydrofolate + glycine + H2O = tetrahydrofolate + L-serine [RN:R00945] Gene Name: SHMT2 Uniprot ID: Q5BJF5 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Serine hydroxymethyltransferase Reactions: 5,10-methylenetetrahydrofolate + glycine + H2O = tetrahydrofolate + L-serine [RN:R00945] Gene Name: DKFZp686P09201 Uniprot ID: Q5HYG8 Protein Sequence: FASTA Gene Sequence: FASTA
Name: N-arachidonyl glycine receptor Reactions: Gene Name: GPR18 Uniprot ID: Q14330 Protein Sequence: FASTA Gene Sequence: FASTA

Transporters
Name: Proton-coupled amino acid transporter 1 Reactions: Gene Name: SLC36A1 Uniprot ID: Q7Z2H8 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Monocarboxylate transporter 10 Reactions: Gene Name: SLC16A10 Uniprot ID: Q8TF71 Protein Sequence: FASTA Gene Sequence: FASTA

This project is supported by Genome Alberta & Genome Canada, a not-for-profit organization that is leading Canada's national genomics strategy with $600 million in funding from the federal government.