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Record Information
Version3.6
Creation Date2005-11-16 15:48:42 UTC
Update Date2014-06-11 20:29:52 UTC
HMDB IDHMDB00123
Secondary Accession NumbersNone
Metabolite Identification
Common NameGlycine
DescriptionGlycine 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
Thumb
Synonyms
  1. 2-Aminoacetate
  2. 2-Aminoacetic acid
  3. Aciport
  4. Amino-Acetate
  5. Amino-Acetic acid
  6. Aminoacetate
  7. Aminoacetic acid
  8. Aminoethanoate
  9. Aminoethanoic acid
  10. Glicoamin
  11. Glycocoll
  12. Glycolixir
  13. Glycosthene
  14. Gyn-Hydralin
  15. Padil
Chemical FormulaC2H5NO2
Average Molecular Weight75.0666
Monoisotopic Molecular Weight75.032028409
IUPAC Name2-aminoacetic acid
Traditional IUPAC Nameglycine
CAS Registry Number56-40-6
SMILES
NCC(O)=O
InChI Identifier
InChI=1S/C2H5NO2/c3-1-2(4)5/h1,3H2,(H,4,5)
InChI KeyDHMQDGOQFOQNFH-UHFFFAOYSA-N
Chemical Taxonomy
KingdomOrganic Compounds
Super ClassAmino Acids, Peptides, and Analogues
ClassAmino Acids and Derivatives
Sub ClassAlpha 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 ParentAlpha Amino Acids and Derivatives
Ontology
StatusDetected and Quantified
Origin
  • Endogenous
Biofunction
  • Non-essential amino acid
ApplicationNot Available
Cellular locations
  • Extracellular
  • Mitochondria
  • Lysosome
  • Peroxisome
Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting Point262.2 °CNot Available
Boiling PointNot AvailableNot Available
Water Solubility249.0 mg/mLNot Available
LogP-3.21HANSCH,C ET AL. (1995)
Predicted Properties
PropertyValueSource
Water Solubility552 g/LALOGPS
logP-3.3ALOGPS
logP-3.4ChemAxon
logS0.87ALOGPS
pKa (Strongest Acidic)2.31ChemAxon
pKa (Strongest Basic)9.24ChemAxon
Physiological Charge0ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count2ChemAxon
Polar Surface Area63.32ChemAxon
Rotatable Bond Count1ChemAxon
Refractivity16ChemAxon
Polarizability6.65ChemAxon
Spectra
SpectraGC-MSMS/MSLC-MS1D NMR2D NMR
Biological Properties
Cellular Locations
  • Extracellular
  • Mitochondria
  • Lysosome
  • Peroxisome
Biofluid Locations
  • Bile
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Saliva
  • Urine
Tissue Location
  • Bladder
  • Brain
  • Epidermis
  • Fibroblasts
  • Intestine
  • Kidney
  • Myelin
  • Neuron
  • Pancreas
  • Placenta
  • Platelet
  • Prostate
  • Spleen
  • Stratum Corneum
  • Thyroid Gland
Pathways
NameSMPDB LinkKEGG Link
Alanine MetabolismSMP00055map00250
Ammonia RecyclingSMP00009map00910
Bile Acid BiosynthesisSMP00035map00120
Carnitine SynthesisSMP00465Not Available
Glutathione MetabolismSMP00015map00480
Glycine and Serine MetabolismSMP00004map00260
Methionine MetabolismSMP00033map00270
Porphyrin MetabolismSMP00024map00860
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BileDetected and Quantified>10 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified242.0 +/- 44.0 uMAdult (>18 years old)MaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified258.0 +/- 64.0 uMAdult (>18 years old)FemaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified329.9 +/- 105.6 uMAdult (>18 years old)Not SpecifiedNormal details
BloodDetected and Quantified280.0 (140.0-420.0) uMNewborn (0-30 days old)BothNormal details
BloodDetected and Quantified486.0 (232.0-740.0) uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified212.4 +/- 57.4 uMAdult (>18 years old)MaleNormal details
BloodDetected and Quantified230.0 (178.0-282.0) uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified325.4 +/- 126.8 uMAdult (>18 years old)Not SpecifiedNormal details
BloodDetected and Quantified460.0 +/- 275.0 uMNewborn (0-30 days old)Not SpecifiedNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified234.0 +/- 34.0 uMChildren (1-13 years old)MaleNormal
    • Geigy Scientific ...
details
Cerebrospinal Fluid (CSF)Detected and Quantified4.7 +/- 1.5 uMAdult (>18 years old)BothNormal
    • Geigy Scientific ...
details
Cerebrospinal Fluid (CSF)Detected and Quantified9.0 (3.0-19.0) uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified9.5 +/- 4.7 uMAdult (>18 years old)Not SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified8.2 +/- 3.0 uMAdult (>18 years old)Not SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified6.1 +/- 1.4 uMAdult (>18 years old)Not SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified4.63 +/- 0.74 uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified8.30 (5.88-10.7) uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified11 +/- 4  uMNot SpecifiedNot SpecifiedNormal details
SalivaDetected and Quantified1-472 uMAdult (>18 years old)Male
normal
details
SalivaDetected and Quantified1-94 uMAdult (>18 years old)Male
normal
details
SalivaDetected and Quantified>10 uMAdult (>18 years old)BothNormal details
UrineDetected and Quantified75.7 +/- 51.7 umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified165.0 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified151(48-233) umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified119.95-599.74 umol/mmol creatinineAdult (>18 years old)BothNormal
    • David F. Putnam C...
details
UrineDetected and Quantified151.0 (233-248) umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified513.54 +/- 360.61 umol/mmol creatinineInfant (0-1 year old)BothNormal details
UrineDetected and Quantified106 (44-300) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified71.23 umol/mmol creatinineAdult (>18 years old)MaleNormal
    • Shaykhutdinov RA,...
details
UrineDetected and Quantified128.1 +/- 65.5 umol/mmol creatinineAdult (>18 years old)MaleNormal details
UrineDetected and Quantified187.6 +/- 92.3 umol/mmol creatinineAdult (>18 years old)FemaleNormal details
UrineDetected and Quantified15.0 (10.0-23.0) umol/mmol creatinineNewborn (0-30 days old)BothNormal
    • Geigy Scientific ...
    • West Cadwell, N.J...
    • Basel, Switzerlan...
details
UrineDetected and Quantified30.0 +/- 19.0 umol/mmol creatinineChildren (1-13 years old)BothNormal
    • Geigy Scientific ...
    • West Cadwell, N.J...
    • Basel, Switzerlan...
details
UrineDetected and Quantified92.0 +/- 39.0 umol/mmol creatinineAdult (>18 years old)MaleNormal
    • Geigy Scientific ...
    • West Cadwell, N.J...
    • Basel, Switzerlan...
details
UrineDetected and Quantified139.0 +/- 86.0 umol/mmol creatinineAdult (>18 years old)FemaleNormal
    • Geigy Scientific ...
    • West Cadwell, N.J...
    • Basel, Switzerlan...
details
UrineDetected and Quantified136.48 +/- 77.27 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified101 (37.0-250.6) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified118.9 umol/mmol creatinineAdult (>18 years old)BothNormal details
Abnormal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified234.9 +/- 181.1 uMAdult (>18 years old)Not SpecifiedHeart Transplant details
BloodDetected and Quantified1090.0 (780.0-1400.0) uMNewborn (0-30 days old)BothNon-ketotic hyperglycinemia details
BloodDetected and Quantified1000.0 (800.0-1200.0) uMChildren (1-13 years old)BothD-Glyceric acidura details
BloodDetected and Quantified210.0 (203.0-217.0) uMAdult (>18 years old)BothRefractory localization-related epilepsy (RLE) details
BloodDetected and Quantified354.0 (309.0-399.0) uMChildren (1-13 years old)BothJuvenile myoclonic epilepsy (JME) details
BloodDetected and Quantified0.153 +/- 0.012 uMElderly (>65 years old)BothAlzheimer's disease details
Cerebrospinal Fluid (CSF)Detected and Quantified9.7 +/- 4.9 uMChildren (1-13 years old)Not SpecifiedLeukemia details
Cerebrospinal Fluid (CSF)Detected and Quantified13.4 +/- 6.4 uMChildren (1-13 years old)Not Specified
Leukemia
details
Cerebrospinal Fluid (CSF)Detected and Quantified9.0405 (8.716-9.623) uMNot SpecifiedNot Specifiedcontrol details
Cerebrospinal Fluid (CSF)Detected and Quantified4.5 +/- 0.75 uMAdult (>18 years old)BothSchizophrenia details
Cerebrospinal Fluid (CSF)Detected and Quantified0.28 +/- 0.12 uMAdult (>18 years old)BothAlzheimer's disease details
UrineDetected and Quantified3000.0 (1000.0-5000.0) umol/mmol creatinineChildren (1-13 years old)BothCarbamoyl phosphate synthetase deficiency details
UrineDetected and Quantified0.0024 - 0.0339 umol/mmol creatinineAdult (>18 years old)BothADPKD details
UrineDetected and Quantified84.0 +/- 67.0 umol/mmol creatinineAdult (>18 years old)BothLung cancer details
UrineDetected and Quantified0.010 +/- 0.001 umol/mmol creatinineAdult (>18 years old)BothAlzheimer's disease details
UrineDetected and Quantified178.2 +/- 6.4 umol/mmol creatinineAdult (>18 years old)Both3-Hydroxy-3- methylglutaryl-CoA lyase (HL) deficency details
UrineDetected and Quantified135.8 +/- 5.8 umol/mmol creatinineAdult (>18 years old)Both3-Hydroxy-3- methylglutaryl-CoA lyase (HL) deficency details
UrineDetected and Quantified53.1 +/- 1.8 umol/mmol creatinineAdult (>18 years old)BothArgininosuccinic aciduria (ASL) details
UrineDetected and Quantified585.5 +/- 32.1 umol/mmol creatinineAdult (>18 years old)BothPropionic acidemia details
UrineDetected and Quantified550.00 (100.00-1000.00) umol/mmol creatinineChildren (1-13 years old)BothIminoglycinuria details
UrineDetected and Quantified2040.3 +/- 202.6 umol/mmol creatinineAdult (>18 years old)BothPropionic acidemia details
UrineDetected and Quantified49.9 +/- 1.3 umol/mmol creatinineAdult (>18 years old)BothTyrosinemia I details
UrineDetected and Quantified176.2 +/- 9.2 umol/mmol creatinineAdult (>18 years old)BothTyrosinemia I details
UrineDetected and Quantified82.2 +/- 4.1 umol/mmol creatinineAdult (>18 years old)BothPhenylketonuria details
UrineDetected and Quantified304.8 +/- 11.3 umol/mmol creatinineAdult (>18 years old)BothPhenylketonuria details
UrineDetected and Quantified233.7 +/- 10.5 umol/mmol creatinineAdult (>18 years old)BothMaple syrup urine disease details
UrineDetected and Quantified3205.1 +/- 83.4 umol/mmol creatinineAdult (>18 years old)BothAminoaciduria details
Associated Disorders and Diseases
Disease References
Alzheimer's disease
  1. 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
Carbamoyl Phosphate Synthetase Deficiency
  1. MetaGene
D-Glyceric acidemia
  1. 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
  2. MetaGene
Schizophrenia
  1. 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
Juvenile myoclonic epilepsy
  1. 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
Leukemia
  1. 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
Lung Cancer
  1. Stretch C, Eastman T, Mandal R, Eisner R, Wishart DS, Mourtzakis M, Prado CM, Damaraju S, Ball RO, Greiner R, Baracos VE: Prediction of skeletal muscle and fat mass in patients with advanced cancer using a metabolomic approach. J Nutr. 2012 Jan;142(1):14-21. doi: 10.3945/jn.111.147751. Epub 2011 Dec 7. Pubmed: 22157537
Refractory localization-related epilepsy
  1. 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
Iminoglycinuria
  1. MetaGene
Associated OMIM IDs
DrugBank IDDB00145
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite ID1062
FoodDB IDFDB000484
KNApSAcK IDC00001361
Chemspider ID730
KEGG Compound IDC00037
BioCyc IDGLY
BiGG ID33610
Wikipedia LinkGlycine
NuGOwiki LinkHMDB00123
Metagene LinkHMDB00123
METLIN ID20
PubChem Compound750
PDB IDGLY
ChEBI ID15428
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
  1. 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
  2. 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
  3. 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
  4. 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
  5. 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
  6. 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
  7. 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
  8. 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
  9. 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
  10. 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
  11. 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
  12. 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
  13. 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
  14. 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
  15. 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
  16. 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
  17. 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
  18. 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
  19. 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
  20. 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
  21. Van Hove JL, Vande Kerckhove K, Hennermann JB, Mahieu V, Declercq P, Mertens S, De Becker M, Kishnani PS, Jaeken J: Benzoate treatment and the glycine index in nonketotic hyperglycinaemia. J Inherit Metab Dis. 2005;28(5):651-63. Pubmed: 16151895

Enzymes

General function:
Involved in 5-aminolevulinate synthase activity
Specific function:
Not Available
Gene Name:
ALAS2
Uniprot ID:
P22557
Molecular weight:
64632.86
Reactions
Succinyl-CoA + Glycine → 5-Aminolevulinic acid + Coenzyme A + CO(2)details
Succinyl-CoA + Glycine → 5-Aminolevulinic acid + Coenzyme A + Carbon dioxidedetails
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. Pubmed: 17139284
  2. Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. Pubmed: 17016423
  3. Hungerer C, Troup B, Romling U, Jahn D: Regulation of the hemA gene during 5-aminolevulinic acid formation in Pseudomonas aeruginosa. J Bacteriol. 1995 Mar;177(6):1435-43. Pubmed: 7883699
  4. Shoolingin-Jordan PM, Al-Daihan S, Alexeev D, Baxter RL, Bottomley SS, Kahari ID, Roy I, Sarwar M, Sawyer L, Wang SF: 5-Aminolevulinic acid synthase: mechanism, mutations and medicine. Biochim Biophys Acta. 2003 Apr 11;1647(1-2):361-6. Pubmed: 12686158
  5. Munakata H, Yamagami T, Nagai T, Yamamoto M, Hayashi N: Purification and structure of rat erythroid-specific delta-aminolevulinate synthase. J Biochem. 1993 Jul;114(1):103-11. Pubmed: 8407861
General function:
Involved in 5-aminolevulinate synthase activity
Specific function:
Not Available
Gene Name:
ALAS1
Uniprot ID:
P13196
Molecular weight:
70580.325
Reactions
Succinyl-CoA + Glycine → 5-Aminolevulinic acid + Coenzyme A + CO(2)details
Succinyl-CoA + Glycine → 5-Aminolevulinic acid + Coenzyme A + Carbon dioxidedetails
References
  1. Turbeville TD, Zhang J, Hunter GA, Ferreira GC: Histidine 282 in 5-aminolevulinate synthase affects substrate binding and catalysis. Biochemistry. 2007 May 22;46(20):5972-81. Epub 2007 May 1. Pubmed: 17469798
  2. He XM, Zhou J, Cheng Y, Fan J: [Purification and production of the extracellular 5-aminolevulinate from recombiniant Escherichia coli expressing yeast ALAS]. Sheng Wu Gong Cheng Xue Bao. 2007 May;23(3):520-4. Pubmed: 17578005
General function:
Involved in glycine C-acetyltransferase activity
Specific function:
Not Available
Gene Name:
GCAT
Uniprot ID:
O75600
Molecular weight:
47973.79
Reactions
Acetyl-CoA + Glycine → Coenzyme A + L-2-Amino-3-oxobutanoic aciddetails
Acetyl-CoA + Glycine → Coenzyme A + L-2-Amino-3-oxobutanoic aciddetails
References
  1. Bashir Q, Rashid N, Akhtar M: Mechanism and substrate stereochemistry of 2-amino-3-oxobutyrate CoA ligase: implications for 5-aminolevulinate synthase and related enzymes. Chem Commun (Camb). 2006 Dec 28;(48):5065-7. Epub 2006 Oct 13. Pubmed: 17146529
General function:
Involved in oxidoreductase activity
Specific function:
Metabolizes sarcosine, L-pipecolic acid and L-proline.
Gene Name:
PIPOX
Uniprot ID:
Q9P0Z9
Molecular weight:
44065.515
Reactions
Sarcosine + Water + Oxygen → Glycine + Formaldehyde + Hydrogen peroxidedetails
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. Pubmed: 17139284
  2. Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. Pubmed: 17016423
General function:
Involved in D-amino-acid oxidase activity
Specific function:
Regulates the level of the neuromodulator D-serine in the brain. Has high activity towards D-DOPA and contributes to dopamine synthesis. Could act as a detoxifying agent which removes D-amino acids accumulated during aging. Acts on a variety of D-amino acids with a preference for those having small hydrophobic side chains followed by those bearing polar, aromatic, and basic groups. Does not act on acidic amino acids.
Gene Name:
DAO
Uniprot ID:
P14920
Molecular weight:
39473.75
Reactions
Glycine + Water + Oxygen → Glyoxylic acid + Ammonia + Hydrogen peroxidedetails
General function:
Involved in catalytic activity
Specific function:
Not Available
Gene Name:
GART
Uniprot ID:
P22102
Molecular weight:
107766.295
Reactions
Adenosine triphosphate + 5-Phosphoribosylamine + Glycine → ADP + Phosphoric acid + Glycineamideribotidedetails
Adenosine triphosphate + 5-Phosphoribosylamine + Glycine → ADP + Phosphoric acid + Glycineamideribotidedetails
General function:
Involved in transaminase activity
Specific function:
Can metabolize asymmetric dimethylarginine (ADMA) via transamination to alpha-keto-delta-(NN-dimethylguanidino) valeric acid (DMGV). ADMA is a potent inhibitor of nitric-oxide (NO) synthase, and this activity provides mechanism through which the kidney regulates blood pressure.
Gene Name:
AGXT2
Uniprot ID:
Q9BYV1
Molecular weight:
57155.905
Reactions
L-Alanine + Glyoxylic acid → Pyruvic acid + Glycinedetails
Glycine + Oxoglutaric acid → Glyoxylic acid + L-Glutamic aciddetails
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. Pubmed: 17139284
  2. Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. Pubmed: 17016423
  3. Baker PR, Cramer SD, Kennedy M, Assimos DG, Holmes RP: Glycolate and glyoxylate metabolism in HepG2 cells. Am J Physiol Cell Physiol. 2004 Nov;287(5):C1359-65. Epub 2004 Jul 7. Pubmed: 15240345
  4. Takada Y, Mori T, Noguchi T: The effect of vitamin B6 deficiency on alanine: glyoxylate aminotransferase isoenzymes in rat liver. Arch Biochem Biophys. 1984 Feb 15;229(1):1-6. Pubmed: 6703688
General function:
Involved in folic acid binding
Specific function:
Catalyzes the methylation of glycine by using S-adenosylmethionine (AdoMet) to form N-methylglycine (sarcosine) with the concomitant production of S-adenosylhomocysteine (AdoHcy). Possible crucial role in the regulation of tissue concentration of AdoMet and of metabolism of methionine.
Gene Name:
GNMT
Uniprot ID:
Q14749
Molecular weight:
32742.0
Reactions
S-Adenosylmethionine + Glycine → S-Adenosylhomocysteine + Sarcosinedetails
References
  1. Soriano A, Castillo R, Christov C, Andres J, Moliner V, Tunon I: Catalysis in glycine N-methyltransferase: testing the electrostatic stabilization and compression hypothesis. Biochemistry. 2006 Dec 19;45(50):14917-25. Pubmed: 17154529
  2. Luka Z, Pakhomova S, Loukachevitch LV, Egli M, Newcomer ME, Wagner C: 5-methyltetrahydrofolate is bound in intersubunit areas of rat liver folate-binding protein glycine N-methyltransferase. J Biol Chem. 2007 Feb 9;282(6):4069-75. Epub 2006 Dec 7. Pubmed: 17158459
  3. Velichkova P, Himo F: Methyl transfer in glycine N-methyltransferase. A theoretical study. J Phys Chem B. 2005 Apr 28;109(16):8216-9. Pubmed: 16851960
General function:
Involved in proteolysis
Specific function:
Broad specificity aminopeptidase. Plays a role in the final digestion of peptides generated from hydrolysis of proteins by gastric and pancreatic proteases. May play a critical role in the pathogenesis of cholesterol gallstone disease. May be involved in the metabolism of regulatory peptides of diverse cell types, responsible for the processing of peptide hormones, such as angiotensin III and IV, neuropeptides, and chemokines. Found to cleave antigen peptides bound to major histocompatibility complex class II molecules of presenting cells and to degrade neurotransmitters at synaptic junctions. Is also implicated as a regulator of IL-8 bioavailability in the endometrium, and therefore may contribute to the regulation of angiogenesis. Is used as a marker for acute myeloid leukemia and plays a role in tumor invasion. In case of human coronavirus 229E (HCoV-229E) infection, serves as receptor for HCoV-229E spike glycoprotein. Mediates as well human cytomegalovirus (HCMV) infection.
Gene Name:
ANPEP
Uniprot ID:
P15144
Molecular weight:
Not Available
Reactions
Cysteinylglycine + Water → L-Cysteine + Glycinedetails
R-S-Cysteinylglycine + Water → S-Substituted L-cysteine + Glycinedetails
General function:
Amino acid transport and metabolism
Specific function:
Catalyzes the biosynthesis of guanidinoacetate, the immediate precursor of creatine. Creatine plays a vital role in energy metabolism in muscle tissues. May play a role in embryonic and central nervous system development. May be involved in the response to heart failure by elevating local creatine synthesis.
Gene Name:
GATM
Uniprot ID:
P50440
Molecular weight:
48455.01
Reactions
L-Arginine + Glycine → Ornithine + Guanidoacetic aciddetails
References
  1. Wang L, Zhang Y, Shao M, Zhang H: Spatiotemporal expression of the creatine metabolism related genes agat, gamt and ct1 during zebrafish embryogenesis. Int J Dev Biol. 2007;51(3):247-53. Pubmed: 17486546
  2. Dutta U, Cohenford MA, Guha M, Dain JA: Non-enzymatic interactions of glyoxylate with lysine, arginine, and glucosamine: a study of advanced non-enzymatic glycation like compounds. Bioorg Chem. 2007 Feb;35(1):11-24. Epub 2006 Sep 12. Pubmed: 16970975
General function:
Involved in thiolester hydrolase activity
Specific function:
Involved in bile acid metabolism. In liver hepatocytes catalyzes the second step in the conjugation of C24 bile acids (choloneates) to glycine and taurine before excretion into bile canaliculi. The major components of bile are cholic acid and chenodeoxycholic acid. In a first step the bile acids are converted to an acyl-CoA thioester, either in peroxisomes (primary bile acids deriving from the cholesterol pathway), or cytoplasmic at the endoplasmic reticulum (secondary bile acids). May catalyze the conjugation of primary or secondary bile acids, or both. The conjugation increases the detergent properties of bile acids in the intestine, which facilitates lipid and fat-soluble vitamin absorption. In turn, bile acids are deconjugated by bacteria in the intestine and are recycled back to the liver for reconjugation (secondary bile acids). May also act as an acyl-CoA thioesterase that regulates intracellular levels of free fatty acids. In vitro, catalyzes the hydrolysis of long- and very long-chain saturated acyl-CoAs to the free fatty acid and coenzyme A (CoASH), and conjugates glycine to these acyl-CoAs.
Gene Name:
BAAT
Uniprot ID:
Q14032
Molecular weight:
46298.865
Reactions
Choloyl-CoA + Glycine → Coenzyme A + Glycocholic aciddetails
Chenodeoxycholoyl-CoA + Glycine → Chenodeoxycholic acid glycine conjugate + Coenzyme Adetails
References
  1. Styles NA, Falany JL, Barnes S, Falany CN: Quantification and regulation of the subcellular distribution of bile acid coenzyme A:amino acid N-acyltransferase activity in rat liver. J Lipid Res. 2007 Jun;48(6):1305-15. Epub 2007 Mar 22. Pubmed: 17379925
  2. Visser WF, van Roermund CW, Ijlst L, Waterham HR, Wanders RJ: Demonstration of bile acid transport across the mammalian peroxisomal membrane. Biochem Biophys Res Commun. 2007 Jun 1;357(2):335-40. Epub 2007 Mar 26. Pubmed: 17416343
  3. Pellicoro A, van den Heuvel FA, Geuken M, Moshage H, Jansen PL, Faber KN: Human and rat bile acid-CoA:amino acid N-acyltransferase are liver-specific peroxisomal enzymes: implications for intracellular bile salt transport. Hepatology. 2007 Feb;45(2):340-8. Pubmed: 17256745
  4. Nakamura K, Morrison SF: Central efferent pathways mediating skin cooling-evoked sympathetic thermogenesis in brown adipose tissue. Am J Physiol Regul Integr Comp Physiol. 2007 Jan;292(1):R127-36. Epub 2006 Aug 24. Pubmed: 16931649
General function:
Involved in nucleotide binding
Specific function:
Catalyzes the attachment of glycine to tRNA(Gly). Is also able produce diadenosine tetraphosphate (Ap4A), a universal pleiotropic signaling molecule needed for cell regulation pathways, by direct condensation of 2 ATPs.
Gene Name:
GARS
Uniprot ID:
P41250
Molecular weight:
83164.83
Reactions
Adenosine triphosphate + Glycine + tRNA(Gly) → Adenosine monophosphate + Pyrophosphate + glycyl-tRNA(Gly)details
Adenosine triphosphate + Glycine + tRNA(Gly) → Adenosine monophosphate + Pyrophosphate + Glycyl-tRNA(Gly)details
References
  1. Okamoto K, Kuno A, Hasegawa T: Recognition sites of glycine tRNA for glycyl-tRNA synthetase from hyperthermophilic archaeon, Aeropyrum pernix K1. Nucleic Acids Symp Ser (Oxf). 2005;(49):299-300. Pubmed: 17150752
  2. Tsang SW, Vinters HV, Cummings JL, Wong PT, Chen CP, Lai MK: Alterations in NMDA receptor subunit densities and ligand binding to glycine recognition sites are associated with chronic anxiety in Alzheimer's disease. Neurobiol Aging. 2008 Oct;29(10):1524-32. Epub 2007 Apr 11. Pubmed: 17433503
  3. Scherer SS: Inherited neuropathies: new genes don't fit old models. Neuron. 2006 Sep 21;51(6):672-4. Pubmed: 16982409
  4. Cader MZ, Ren J, James PA, Bird LE, Talbot K, Stammers DK: Crystal structure of human wildtype and S581L-mutant glycyl-tRNA synthetase, an enzyme underlying distal spinal muscular atrophy. FEBS Lett. 2007 Jun 26;581(16):2959-64. Epub 2007 May 29. Pubmed: 17544401
  5. Antonellis A, Lee-Lin SQ, Wasterlain A, Leo P, Quezado M, Goldfarb LG, Myung K, Burgess S, Fischbeck KH, Green ED: Functional analyses of glycyl-tRNA synthetase mutations suggest a key role for tRNA-charging enzymes in peripheral axons. J Neurosci. 2006 Oct 11;26(41):10397-406. Pubmed: 17035524
General function:
Involved in aminomethyltransferase activity
Specific function:
The glycine cleavage system catalyzes the degradation of glycine.
Gene Name:
AMT
Uniprot ID:
P48728
Molecular weight:
43945.65
Reactions
Glycine + Tetrahydrofolic acid + NAD → 5,10-Methylene-THF + Ammonia + Carbon dioxide + NADH + Hydrogen Iondetails
General function:
Involved in metabolic process
Specific function:
Not Available
Gene Name:
AGXT
Uniprot ID:
P21549
Molecular weight:
43009.535
Reactions
L-Alanine + Glyoxylic acid → Pyruvic acid + Glycinedetails
Glycine + Oxoglutaric acid → Glyoxylic acid + L-Glutamic aciddetails
L-Serine + Glyoxylic acid → Hydroxypyruvic acid + Glycinedetails
References
  1. Ricoult C, Echeverria LO, Cliquet JB, Limami AM: Characterization of alanine aminotransferase (AlaAT) multigene family and hypoxic response in young seedlings of the model legume Medicago truncatula. J Exp Bot. 2006;57(12):3079-89. Epub 2006 Aug 9. Pubmed: 16899523
  2. Han Q, Robinson H, Gao YG, Vogelaar N, Wilson SR, Rizzi M, Li J: Crystal structures of Aedes aegypti alanine glyoxylate aminotransferase. J Biol Chem. 2006 Dec 1;281(48):37175-82. Epub 2006 Sep 21. Pubmed: 16990263
  3. Lu TC, Ko YZ, Huang HW, Hung YC, Lin YC, Peng WH: Analgesic and anti-inflammatory activities of aqueous extract from Glycine tomentella root in mice. J Ethnopharmacol. 2007 Aug 15;113(1):142-8. Epub 2007 May 31. Pubmed: 17616291
General function:
Involved in catalytic activity
Specific function:
Not Available
Gene Name:
GSS
Uniprot ID:
P48637
Molecular weight:
52384.325
Reactions
Adenosine triphosphate + Gamma-Glutamylcysteine + Glycine → ADP + Phosphoric acid + Glutathionedetails
References
  1. Herrera K, Cahoon RE, Kumaran S, Jez J: Reaction mechanism of glutathione synthetase from Arabidopsis thaliana: site-directed mutagenesis of active site residues. J Biol Chem. 2007 Jun 8;282(23):17157-65. Epub 2007 Apr 22. Pubmed: 17452339
  2. Ducruix C, Junot C, Fievet JB, Villiers F, Ezan E, Bourguignon J: New insights into the regulation of phytochelatin biosynthesis in A. thaliana cells from metabolite profiling analyses. Biochimie. 2006 Nov;88(11):1733-42. Epub 2006 Sep 7. Pubmed: 16996193
  3. Ristoff E, Larsson A: Inborn errors in the metabolism of glutathione. Orphanet J Rare Dis. 2007 Mar 30;2:16. Pubmed: 17397529
  4. Pai CH, Chiang BY, Ko TP, Chou CC, Chong CM, Yen FJ, Chen S, Coward JK, Wang AH, Lin CH: Dual binding sites for translocation catalysis by Escherichia coli glutathionylspermidine synthetase. EMBO J. 2006 Dec 13;25(24):5970-82. Epub 2006 Nov 23. Pubmed: 17124497
  5. Janaky R, Dohovics R, Saransaari P, Oja SS: Modulation of [3H]dopamine release by glutathione in mouse striatal slices. Neurochem Res. 2007 Aug;32(8):1357-64. Epub 2007 Mar 31. Pubmed: 17401648
General function:
Involved in catalytic activity
Specific function:
Contributes to the de novo mitochondrial thymidylate biosynthesis pathway. Required to prevent uracil accumulation in mtDNA. Interconversion of serine and glycine. Associates with mitochondrial DNA.
Gene Name:
SHMT2
Uniprot ID:
P34897
Molecular weight:
54862.125
Reactions
5,10-Methylene-THF + Glycine + Water → Tetrahydrofolic acid + L-Serinedetails
L-Serine + 5,6,7,8-Tetrahydromethanopterin → 5,10-Methylenetetrahydromethanopterin + Glycine + Waterdetails
References
  1. Gagnon D, Foucher A, Girard I, Ouellette M: Stage specific gene expression and cellular localization of two isoforms of the serine hydroxymethyltransferase in the protozoan parasite Leishmania. Mol Biochem Parasitol. 2006 Nov;150(1):63-71. Epub 2006 Jul 13. Pubmed: 16876889
  2. Vatsyayan R, Roy U: Molecular cloning and biochemical characterization of Leishmania donovani serine hydroxymethyltransferase. Protein Expr Purif. 2007 Apr;52(2):433-40. Epub 2006 Oct 26. Pubmed: 17142057
  3. Rajinikanth M, Harding SA, Tsai CJ: The glycine decarboxylase complex multienzyme family in Populus. J Exp Bot. 2007;58(7):1761-70. Epub 2007 Mar 12. Pubmed: 17355947
  4. Mukherjee M, Sievers SA, Brown MT, Johnson PJ: Identification and biochemical characterization of serine hydroxymethyl transferase in the hydrogenosome of Trichomonas vaginalis. Eukaryot Cell. 2006 Dec;5(12):2072-8. Epub 2006 Sep 15. Pubmed: 16980404
  5. Chang WN, Tsai JN, Chen BH, Huang HS, Fu TF: Serine hydroxymethyltransferase isoforms are differentially inhibited by leucovorin: characterization and comparison of recombinant zebrafish serine hydroxymethyltransferases. Drug Metab Dispos. 2007 Nov;35(11):2127-37. Epub 2007 Jul 30. Pubmed: 17664250
General function:
Involved in oxidoreductase activity
Specific function:
Not Available
Gene Name:
SARDH
Uniprot ID:
Q9UL12
Molecular weight:
101035.985
Reactions
Sarcosine + Water + electron-transfer flavoprotein → Glycine + Formaldehyde + reduced electron-transfer flavoproteindetails
Sarcosine + Electron-transferring flavoprotein + Water → Glycine + Formaldehyde + Reduced electron-transferring flavoproteindetails
General function:
Involved in catalytic activity
Specific function:
Interconversion of serine and glycine.
Gene Name:
SHMT1
Uniprot ID:
P34896
Molecular weight:
53082.18
Reactions
5,10-Methylene-THF + Glycine + Water → Tetrahydrofolic acid + L-Serinedetails
L-Serine + 5,6,7,8-Tetrahydromethanopterin → 5,10-Methylenetetrahydromethanopterin + Glycine + Waterdetails
References
  1. Vatsyayan R, Roy U: Molecular cloning and biochemical characterization of Leishmania donovani serine hydroxymethyltransferase. Protein Expr Purif. 2007 Apr;52(2):433-40. Epub 2006 Oct 26. Pubmed: 17142057
  2. Rajinikanth M, Harding SA, Tsai CJ: The glycine decarboxylase complex multienzyme family in Populus. J Exp Bot. 2007;58(7):1761-70. Epub 2007 Mar 12. Pubmed: 17355947
  3. Chang WN, Tsai JN, Chen BH, Huang HS, Fu TF: Serine hydroxymethyltransferase isoforms are differentially inhibited by leucovorin: characterization and comparison of recombinant zebrafish serine hydroxymethyltransferases. Drug Metab Dispos. 2007 Nov;35(11):2127-37. Epub 2007 Jul 30. Pubmed: 17664250
  4. Nijhout HF, Reed MC, Lam SL, Shane B, Gregory JF 3rd, Ulrich CM: In silico experimentation with a model of hepatic mitochondrial folate metabolism. Theor Biol Med Model. 2006 Dec 6;3:40. Pubmed: 17150100
  5. Gagnon D, Foucher A, Girard I, Ouellette M: Stage specific gene expression and cellular localization of two isoforms of the serine hydroxymethyltransferase in the protozoan parasite Leishmania. Mol Biochem Parasitol. 2006 Nov;150(1):63-71. Epub 2006 Jul 13. Pubmed: 16876889
General function:
Involved in lyase activity
Specific function:
The glycine cleavage system catalyzes the degradation of glycine. The P protein binds the alpha-amino group of glycine through its pyridoxal phosphate cofactor; CO(2) is released and the remaining methylamine moiety is then transferred to the lipoamide cofactor of the H protein.
Gene Name:
GLDC
Uniprot ID:
P23378
Molecular weight:
112728.805
Reactions
Glycine + H-protein-lipoyllysine → H-protein-S-aminomethyldihydrolipoyllysine + CO(2)details
Glycine + Lipoylprotein → S-Aminomethyldihydrolipoylprotein + Carbon dioxidedetails
References
  1. Mukherjee M, Brown MT, McArthur AG, Johnson PJ: Proteins of the glycine decarboxylase complex in the hydrogenosome of Trichomonas vaginalis. Eukaryot Cell. 2006 Dec;5(12):2062-71. Pubmed: 17158739
  2. Kanno J, Hutchin T, Kamada F, Narisawa A, Aoki Y, Matsubara Y, Kure S: Genomic deletion within GLDC is a major cause of non-ketotic hyperglycinaemia. J Med Genet. 2007 Mar;44(3):e69. Pubmed: 17361008
  3. Engel N, van den Daele K, Kolukisaoglu U, Morgenthal K, Weckwerth W, Parnik T, Keerberg O, Bauwe H: Deletion of glycine decarboxylase in Arabidopsis is lethal under nonphotorespiratory conditions. Plant Physiol. 2007 Jul;144(3):1328-35. Epub 2007 May 11. Pubmed: 17496108
General function:
Involved in aminopeptidase activity
Specific function:
Presumably involved in the processing and regular turnover of intracellular proteins. Catalyzes the removal of unsubstituted N-terminal amino acids from various peptides.
Gene Name:
LAP3
Uniprot ID:
P28838
Molecular weight:
Not Available
Reactions
Cysteinylglycine + Water → L-Cysteine + Glycinedetails
R-S-Cysteinylglycine + Water → S-Substituted L-cysteine + Glycinedetails
General function:
Involved in ionotropic glutamate receptor activity
Specific function:
NMDA receptor subtype of glutamate-gated ion channels with reduced single-channel conductance, low calcium permeability and low voltage-dependent sensitivity to magnesium. Mediated by glycine
Gene Name:
GRIN3B
Uniprot ID:
O60391
Molecular weight:
112991.0
References
  1. Smothers CT, Woodward JJ: Pharmacological characterization of glycine-activated currents in HEK 293 cells expressing N-methyl-D-aspartate NR1 and NR3 subunits. J Pharmacol Exp Ther. 2007 Aug;322(2):739-48. Epub 2007 May 14. Pubmed: 17502428
General function:
Involved in ionotropic glutamate receptor activity
Specific function:
NMDA receptor subtype of glutamate-gated ion channels possesses high calcium permeability and voltage-dependent sensitivity to magnesium. Activation requires binding of agonist to both types of subunits
Gene Name:
GRIN2A
Uniprot ID:
Q12879
Molecular weight:
165281.2
References
  1. Liu Y, Wong TP, Aarts M, Rooyakkers A, Liu L, Lai TW, Wu DC, Lu J, Tymianski M, Craig AM, Wang YT: NMDA receptor subunits have differential roles in mediating excitotoxic neuronal death both in vitro and in vivo. J Neurosci. 2007 Mar 14;27(11):2846-57. Pubmed: 17360906
  2. Domingues A, Almeida S, da Cruz e Silva EF, Oliveira CR, Rego AC: Toxicity of beta-amyloid in HEK293 cells expressing NR1/NR2A or NR1/NR2B N-methyl-D-aspartate receptor subunits. Neurochem Int. 2007 May;50(6):872-80. Epub 2007 Mar 7. Pubmed: 17403555
  3. Brosnan RJ, Yang L, Milutinovic PS, Zhao J, Laster MJ, Eger EI 2nd, Sonner JM: Ammonia has anesthetic properties. Anesth Analg. 2007 Jun;104(6):1430-3, table of contents. Pubmed: 17513636
  4. Milutinovic PS, Yang L, Cantor RS, Eger EI 2nd, Sonner JM: Anesthetic-like modulation of a gamma-aminobutyric acid type A, strychnine-sensitive glycine, and N-methyl-d-aspartate receptors by coreleased neurotransmitters. Anesth Analg. 2007 Aug;105(2):386-92. Pubmed: 17646495
  5. Gabra BH, Kessler FK, Ritter JK, Dewey WL, Smith FL: Decrease in N-methyl-D-aspartic acid receptor-NR2B subunit levels by intrathecal short-hairpin RNA blocks group I metabotropic glutamate receptor-mediated hyperalgesia. J Pharmacol Exp Ther. 2007 Jul;322(1):186-94. Epub 2007 Apr 3. Pubmed: 17405869
  6. Chen X, Ji ZL, Chen YZ: TTD: Therapeutic Target Database. Nucleic Acids Res. 2002 Jan 1;30(1):412-5. Pubmed: 11752352
General function:
Involved in ionotropic glutamate receptor activity
Specific function:
NMDA receptor subtype of glutamate-gated ion channels with high calcium permeability and voltage-dependent sensitivity to magnesium. Mediated by glycine
Gene Name:
GRIN2C
Uniprot ID:
Q14957
Molecular weight:
134531.1
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. Pubmed: 17139284
  2. Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. Pubmed: 17016423
  3. Widdowson PS, Gyte AJ, Upton R, Wyatt I: Failure of glycine site NMDA receptor antagonists to protect against L-2-chloropropionic acid-induced neurotoxicity highlights the uniqueness of cerebellar NMDA receptors. Brain Res. 1996 Nov 4;738(2):236-42. Pubmed: 8955518
  4. Al-Ghoul WM, Meeker RB, Greenwood RS: Differential expression of five N-methyl-D-aspartate receptor subunit mRNAs in vasopressin and oxytocin neuroendocrine cells. Brain Res Mol Brain Res. 1997 Mar;44(2):262-72. Pubmed: 9073168
  5. Malayev A, Gibbs TT, Farb DH: Inhibition of the NMDA response by pregnenolone sulphate reveals subtype selective modulation of NMDA receptors by sulphated steroids. Br J Pharmacol. 2002 Feb;135(4):901-9. Pubmed: 11861317
General function:
Involved in neurotransmitter:sodium symporter activity
Specific function:
Terminates the action of glycine by its high affinity sodium-dependent reuptake into presynaptic terminals. May play a role in regulation of glycine levels in NMDA receptor-mediated neurotransmission.
Gene Name:
SLC6A9
Uniprot ID:
P48067
Molecular weight:
70582.02
References
  1. Wiles AL, Pearlman RJ, Rosvall M, Aubrey KR, Vandenberg RJ: N-Arachidonyl-glycine inhibits the glycine transporter, GLYT2a. J Neurochem. 2006 Nov;99(3):781-6. Epub 2006 Aug 8. Pubmed: 16899062
  2. Lindsley CW, Wolkenberg SE, Kinney GG: Progress in the preparation and testing of glycine transporter type-1 (GlyT1) inhibitors. Curr Top Med Chem. 2006;6(17):1883-96. Pubmed: 17017963
  3. Igartua I, Solis JM, Bustamante J: Glycine-induced long-term synaptic potentiation is mediated by the glycine transporter GLYT1. Neuropharmacology. 2007 Jun;52(8):1586-95. Epub 2007 Mar 14. Pubmed: 17462677
  4. Sur C, Kinney GG: Glycine transporter 1 inhibitors and modulation of NMDA receptor-mediated excitatory neurotransmission. Curr Drug Targets. 2007 May;8(5):643-9. Pubmed: 17504107
  5. Raiteri L, Stigliani S, Usai C, Diaspro A, Paluzzi S, Milanese M, Raiteri M, Bonanno G: Functional expression of release-regulating glycine transporters GLYT1 on GABAergic neurons and GLYT2 on astrocytes in mouse spinal cord. Neurochem Int. 2008 Jan;52(1-2):103-12. Epub 2007 May 16. Pubmed: 17597258
General function:
Involved in glycine catabolic process
Specific function:
The glycine cleavage system catalyzes the degradation of glycine. The H protein shuttles the methylamine group of glycine from the P protein to the T protein.
Gene Name:
GCSH
Uniprot ID:
P23434
Molecular weight:
Not Available
Reactions
Glycine + Tetrahydrofolic acid + NAD → 5,10-Methylene-THF + Ammonia + Carbon dioxide + NADH + Hydrogen Iondetails
References
  1. Kanno J, Hutchin T, Kamada F, Narisawa A, Aoki Y, Matsubara Y, Kure S: Genomic deletion within GLDC is a major cause of non-ketotic hyperglycinaemia. J Med Genet. 2007 Mar;44(3):e69. Pubmed: 17361008
General function:
Involved in neurotransmitter:sodium symporter activity
Specific function:
Terminates the action of glycine by its high affinity sodium-dependent reuptake into presynaptic terminals. May be responsible for the termination of neurotransmission at strychnine-sensitive glycinergic synapses.
Gene Name:
SLC6A5
Uniprot ID:
Q9Y345
Molecular weight:
87433.13
References
  1. Eulenburg V, Becker K, Gomeza J, Schmitt B, Becker CM, Betz H: Mutations within the human GLYT2 (SLC6A5) gene associated with hyperekplexia. Biochem Biophys Res Commun. 2006 Sep 22;348(2):400-5. Epub 2006 Jul 26. Pubmed: 16884688
General function:
Amino acid transport and metabolism
Specific function:
Involved in the uptake of GABA and glycine into the synaptic vesicles
Gene Name:
SLC32A1
Uniprot ID:
Q9H598
Molecular weight:
57414.6
References
  1. Fujii M, Arata A, Kanbara-Kume N, Saito K, Yanagawa Y, Obata K: Respiratory activity in brainstem of fetal mice lacking glutamate decarboxylase 65/67 and vesicular GABA transporter. Neuroscience. 2007 May 25;146(3):1044-52. Epub 2007 Apr 5. Pubmed: 17418495
  2. Aubrey KR, Rossi FM, Ruivo R, Alboni S, Bellenchi GC, Le Goff A, Gasnier B, Supplisson S: The transporters GlyT2 and VIAAT cooperate to determine the vesicular glycinergic phenotype. J Neurosci. 2007 Jun 6;27(23):6273-81. Pubmed: 17554001
  3. Uchigashima M, Fukaya M, Watanabe M, Kamiya H: Evidence against GABA release from glutamatergic mossy fiber terminals in the developing hippocampus. J Neurosci. 2007 Jul 25;27(30):8088-100. Pubmed: 17652600
General function:
Involved in glycine N-acyltransferase activity
Specific function:
Mitochondrial acyltransferase which transfers an acyl group to the N-terminus of glycine and glutamine, although much less efficiently. Can conjugate numerous substrates to form a variety of N-acylglycines, with a preference for benzoyl-CoA over phenylacetyl-CoA as acyl donors. Thereby detoxify xenobiotics, such as benzoic acid or salicylic acid, and endogenous organic acids, such as isovaleric acid.
Gene Name:
GLYAT
Uniprot ID:
Q6IB77
Molecular weight:
18506.33
Reactions
Acyl-CoA + Glycine → Coenzyme A + N-acylglycinedetails
Benzoyl-CoA + Glycine → Coenzyme A + Hippuric aciddetails
Phenylacetyl-CoA + Glycine → Phenylacetylglycine + Coenzyme Adetails
References
  1. Narasipura SD, Ren P, Dyavaiah M, Auger I, Chaturvedi V, Chaturvedi S: An efficient method for homologous gene reconstitution in Cryptococcus gattii using URA5 auxotrophic marker. Mycopathologia. 2006 Dec;162(6):401-9. Pubmed: 17146584
  2. Zhou CX, Gao Y: Frequent genetic alterations and reduced expression of the Axin1 gene in oral squamous cell carcinoma: involvement in tumor progression and metastasis. Oncol Rep. 2007 Jan;17(1):73-9. Pubmed: 17143481
General function:
Involved in glycine N-acyltransferase activity
Specific function:
Acyltransferase which transfers an acyl group to the N-terminus of glutamine. Can use phenylacetyl-CoA as an acyl donor.
Gene Name:
GLYATL1
Uniprot ID:
Q969I3
Molecular weight:
35100.895
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. Pubmed: 17139284
  2. Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. Pubmed: 17016423
General function:
Involved in glycine N-acyltransferase activity
Specific function:
Mitochondrial acyltransferase which transfers the acyl group to the N-terminus of glycine. Conjugates numerous substrates, such as arachidonoyl-CoA and saturated medium and long-chain acyl-CoAs ranging from chain-length C8:0-CoA to C18:0-CoA, to form a variety of N-acylglycines. Shows a preference for monounsaturated fatty acid oleoyl-CoA (C18:1-CoA) as an acyl donor. Does not exhibit any activity toward C22:6-CoA and chenodeoxycholoyl-CoA, nor toward serine or alanine.
Gene Name:
GLYATL2
Uniprot ID:
Q8WU03
Molecular weight:
34277.055
Reactions
Acyl-CoA + Glycine → Coenzyme A + N-acylglycinedetails
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. Pubmed: 17139284
  2. Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. Pubmed: 17016423
General function:
Involved in ion transport
Specific function:
The glycine receptor is a neurotransmitter-gated ion channel. Binding of glycine to its receptor increases the chloride conductance and thus produces hyperpolarization (inhibition of neuronal firing)
Gene Name:
GLRA1
Uniprot ID:
P23415
Molecular weight:
52623.4
References
  1. Heinze L, Harvey RJ, Haverkamp S, Wassle H: Diversity of glycine receptors in the mouse retina: localization of the alpha4 subunit. J Comp Neurol. 2007 Feb 1;500(4):693-707. Pubmed: 17154252
  2. Eulenburg V, Becker K, Gomeza J, Schmitt B, Becker CM, Betz H: Mutations within the human GLYT2 (SLC6A5) gene associated with hyperekplexia. Biochem Biophys Res Commun. 2006 Sep 22;348(2):400-5. Epub 2006 Jul 26. Pubmed: 16884688
General function:
Involved in ion transport
Specific function:
The glycine receptor is a neurotransmitter-gated ion channel. Binding of glycine to its receptor increases the chloride conductance and thus produces hyperpolarization (inhibition of neuronal firing)
Gene Name:
GLRA2
Uniprot ID:
P23416
Molecular weight:
52001.6
References
  1. Young-Pearse TL, Ivic L, Kriegstein AR, Cepko CL: Characterization of mice with targeted deletion of glycine receptor alpha 2. Mol Cell Biol. 2006 Aug;26(15):5728-34. Pubmed: 16847326
  2. Qi Z, Stephens NR, Spalding EP: Calcium entry mediated by GLR3.3, an Arabidopsis glutamate receptor with a broad agonist profile. Plant Physiol. 2006 Nov;142(3):963-71. Epub 2006 Sep 29. Pubmed: 17012403
  3. Majumdar S, Heinze L, Haverkamp S, Ivanova E, Wassle H: Glycine receptors of A-type ganglion cells of the mouse retina. Vis Neurosci. 2007 Jul-Aug;24(4):471-87. Epub 2007 May 29. Pubmed: 17550639
General function:
Involved in ion transport
Specific function:
The glycine receptor is a neurotransmitter-gated ion channel. Binding of glycine to its receptor increases the chloride conductance and thus produces hyperpolarization (inhibition of neuronal firing)
Gene Name:
GLRA3
Uniprot ID:
O75311
Molecular weight:
53799.8
References
  1. Heinze L, Harvey RJ, Haverkamp S, Wassle H: Diversity of glycine receptors in the mouse retina: localization of the alpha4 subunit. J Comp Neurol. 2007 Feb 1;500(4):693-707. Pubmed: 17154252
  2. Majumdar S, Heinze L, Haverkamp S, Ivanova E, Wassle H: Glycine receptors of A-type ganglion cells of the mouse retina. Vis Neurosci. 2007 Jul-Aug;24(4):471-87. Epub 2007 May 29. Pubmed: 17550639
General function:
Involved in extracellular-glycine-gated chloride channel activity
Specific function:
The glycine receptor is a neurotransmitter-gated ion channel. Binding of glycine to its receptor increases the chloride conductance and thus produces hyperpolarization (inhibition of neuronal firing)
Gene Name:
GLRB
Uniprot ID:
P48167
Molecular weight:
56121.6
References
  1. Eulenburg V, Becker K, Gomeza J, Schmitt B, Becker CM, Betz H: Mutations within the human GLYT2 (SLC6A5) gene associated with hyperekplexia. Biochem Biophys Res Commun. 2006 Sep 22;348(2):400-5. Epub 2006 Jul 26. Pubmed: 16884688
General function:
Involved in transferase activity, transferring nitrogenous groups
Specific function:
Catalyzes the irreversible transamination of the L-tryptophan metabolite L-kynurenine to form kynurenic acid (KA). May catalyze the beta-elimination of S-conjugates and Se-conjugates of L-(seleno)cysteine, resulting in the cleavage of the C-S or C-Se bond (By similarity). Has transaminase activity towards L-kynurenine, tryptophan, phenylalanine, serine, cysteine, methionine, histidine, glutamine and asparagine with glyoxylate as an amino group acceptor (in vitro). Has lower activity with 2-oxoglutarate as amino group acceptor (in vitro) (By similarity).
Gene Name:
CCBL2
Uniprot ID:
Q6YP21
Molecular weight:
51399.855
Reactions
L-Kynurenine + Glyoxylic acid → 4-(2-Aminophenyl)-2,4-dioxobutanoic acid + Glycinedetails
General function:
Involved in catalytic activity
Specific function:
Interconversion of serine and glycine
Gene Name:
Not Available
Uniprot ID:
Q53ET4
Molecular weight:
55973.3
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. Pubmed: 17139284
  2. Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. Pubmed: 17016423
  3. Bouwman RA, Musters RJ, van Beek-Harmsen BJ, de Lange JJ, Lamberts RR, Loer SA, Boer C: Sevoflurane-induced cardioprotection depends on PKC-alpha activation via production of reactive oxygen species. Br J Anaesth. 2007 Nov;99(5):639-45. Epub 2007 Sep 27. Pubmed: 17905752
  4. Chang WN, Tsai JN, Chen BH, Huang HS, Fu TF: Serine hydroxymethyltransferase isoforms are differentially inhibited by leucovorin: characterization and comparison of recombinant zebrafish serine hydroxymethyltransferases. Drug Metab Dispos. 2007 Nov;35(11):2127-37. Epub 2007 Jul 30. Pubmed: 17664250
  5. Kon K, Ikejima K, Okumura K, Aoyama T, Arai K, Takei Y, Lemasters JJ, Sato N: Role of apoptosis in acetaminophen hepatotoxicity. J Gastroenterol Hepatol. 2007 Jun;22 Suppl 1:S49-52. Pubmed: 17567465
General function:
Involved in catalytic activity
Specific function:
Interconversion of serine and glycine
Gene Name:
SHMT2
Uniprot ID:
Q5BJF5
Molecular weight:
52909.3
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. Pubmed: 17139284
  2. Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. Pubmed: 17016423
General function:
Involved in catalytic activity
Specific function:
Interconversion of serine and glycine
Gene Name:
DKFZp686P09201
Uniprot ID:
Q5HYG8
Molecular weight:
53464.4
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. Pubmed: 17139284
  2. Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. Pubmed: 17016423
General function:
Involved in G-protein coupled receptor protein signaling pathway
Specific function:
Receptor for N-arachidonyl glycine. The activity of this receptor is mediated by G proteins which inhibit adenylyl cyclase. May contribute to regulation of the immune system
Gene Name:
GPR18
Uniprot ID:
Q14330
Molecular weight:
38133.3
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. Pubmed: 17139284
  2. Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. Pubmed: 17016423

Transporters

General function:
Amino acid transport and metabolism
Specific function:
Neutral amino acid/proton symporter. Has a pH-dependent electrogenic transport activity for small amino acids such as glycine, alanine and proline. Besides small apolar L-amino acids, it also recognize their D-enantiomers and selected amino acid derivatives such as gamma-aminobutyric acid
Gene Name:
SLC36A1
Uniprot ID:
Q7Z2H8
Molecular weight:
53075.0
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. Pubmed: 17139284
  2. Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. Pubmed: 17016423
  3. Bermingham JR Jr, Pennington J: Organization and expression of the SLC36 cluster of amino acid transporter genes. Mamm Genome. 2004 Feb;15(2):114-25. Pubmed: 15058382
  4. Broer A, Cavanaugh JA, Rasko JE, Broer S: The molecular basis of neutral aminoacidurias. Pflugers Arch. 2006 Jan;451(4):511-7. Epub 2005 Jul 29. Pubmed: 16052352
  5. Metzner L, Kalbitz J, Brandsch M: Transport of pharmacologically active proline derivatives by the human proton-coupled amino acid transporter hPAT1. J Pharmacol Exp Ther. 2004 Apr;309(1):28-35. Epub 2004 Jan 12. Pubmed: 14718599
General function:
Involved in transmembrane transport
Specific function:
Sodium-independent transporter that mediates the update of aromatic acid. Can function as a net efflux pathway for aromatic amino acids in the basosolateral epithelial cells
Gene Name:
SLC16A10
Uniprot ID:
Q8TF71
Molecular weight:
55492.1
References
  1. Kim DK, Kanai Y, Chairoungdua A, Matsuo H, Cha SH, Endou H: Expression cloning of a Na+-independent aromatic amino acid transporter with structural similarity to H+/monocarboxylate transporters. J Biol Chem. 2001 May 18;276(20):17221-8. Epub 2001 Feb 20. Pubmed: 11278508
  2. Kim DK, Kanai Y, Matsuo H, Kim JY, Chairoungdua A, Kobayashi Y, Enomoto A, Cha SH, Goya T, Endou H: The human T-type amino acid transporter-1: characterization, gene organization, and chromosomal location. Genomics. 2002 Jan;79(1):95-103. Pubmed: 11827462