Human Metabolome Database Version 3.5

Showing metabocard for Glycine (HMDB00123)

Record Information
Version 3.5
Creation Date 2005-11-16 08:48:42 -0700
Update Date 2013-05-29 13:24:47 -0600
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 Link_out) 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 Link_out), AMT (OMIM 238310 Link_out), and GCSH (OMIM 238330 Link_out) 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 Link_out).
Structure Thumb
Download: MOL | SDF | PDB | SMILES | InChI
Display: 2D Structure | 3D Structure
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 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 Link_out
Bile Acid Biosynthesis SMP00035 map00120 Link_out
Porphyrin Metabolism SMP00024 map00860 Link_out
Glutathione Metabolism SMP00015 map00480 Link_out
Glycine and Serine Metabolism SMP00004 map00260 Link_out
Alanine Metabolism SMP00055 map00250 Link_out
Methionine Metabolism SMP00033 map00270 Link_out
Carnitine Synthesis SMP00465 Not Available
Normal Concentrations
Biofluid Status Value Age Sex Condition Reference
Bile Detected and Quantified
>10 uM Adult (>18 years old) Both Normal
Blood Detected and Quantified
242.0 +/- 44.0 uM Adult (>18 years old) Male Normal
  • Geigy Scient...
Blood Detected and Quantified
258.0 +/- 64.0 uM Adult (>18 years old) Female Normal
  • Geigy Scient...
Blood Detected and Quantified
329.9 +/- 105.6 uM Adult (>18 years old) Not Specified Normal
Blood Detected and Quantified
280.0 (140.0-420.0) uM Newborn (0-30 days old) Both Normal
Blood Detected and Quantified
486.0 (232.0-740.0) uM Adult (>18 years old) Both Normal
Blood Detected and Quantified
212.4 +/- 57.4 uM Adult (>18 years old) Male Normal
Blood Detected and Quantified
230.0 (178.0-282.0) uM Adult (>18 years old) Both Normal
Blood Detected and Quantified
325.4 +/- 126.8 uM Adult (>18 years old) Not Specified Normal
Blood Detected and Quantified
460.0 +/- 275.0 uM Newborn (0-30 days old) Not Specified Normal
  • Geigy Scient...
Blood Detected and Quantified
234.0 +/- 34.0 uM Children (1-13 year old) Male Normal
  • Geigy Scient...
Cerebrospinal Fluid (CSF) Detected and Quantified
4.7 +/- 1.5 uM Adult (>18 years old) Both Normal
  • Geigy Scient...
Cerebrospinal Fluid (CSF) Detected and Quantified
9.0 (3.0-19.0) uM Adult (>18 years old) Both Normal
Cerebrospinal Fluid (CSF) Detected and Quantified
9.5 +/- 4.7 uM Adult (>18 years old) Not Specified Normal
Cerebrospinal Fluid (CSF) Detected and Quantified
8.2 +/- 3.0 uM Adult (>18 years old) Not Specified Normal
Cerebrospinal Fluid (CSF) Detected and Quantified
6.1 +/- 1.4 uM Adult (>18 years old) Not Specified Normal
Cerebrospinal Fluid (CSF) Detected and Quantified
4.63 +/- 0.74 uM Adult (>18 years old) Both Normal
Cerebrospinal Fluid (CSF) Detected and Quantified
8.30 (5.88-10.7) uM Adult (>18 years old) Both Normal
Cerebrospinal Fluid (CSF) Detected and Quantified
11 +/- 4  uM Not Specified Not Specified Normal
Saliva Detected and Quantified
1-472 uM Adult (>18 years old) Male Comment normal
Saliva Detected and Quantified
1-94 uM Adult (>18 years old) Male Comment normal
Saliva Detected and Quantified
>10 uM Adult (>18 years old) Both Normal
Urine Detected and Quantified
75.7 +/- 51.7 umol/mmol creatinine Adult (>18 years old) Both Comment Normal
Urine Detected but not Quantified
Not Applicable Adult (>18 years old) Male Normal
Urine Detected and Quantified
165.0 umol/mmol creatinine Adult (>18 years old) Both Normal
Urine Detected and Quantified
151(48-233) umol/mmol creatinine Adult (>18 years old) Both Normal
Urine Detected and Quantified
119.95-599.74 umol/mmol creatinine Adult (>18 years old) Both Normal
  • David F. Put...
Urine Detected and Quantified
151.0 (233-248) umol/mmol creatinine Adult (>18 years old) Both Normal
Urine Detected but not Quantified
Not Applicable Adult (>18 years old) Both Normal
Urine Detected and Quantified
513.54 +/- 360.61 umol/mmol creatinine Infant (0-1 year old) Both Normal
Urine Detected and Quantified
106 (44-300) umol/mmol creatinine Adult (>18 years old) Both Comment Normal
Urine Detected and Quantified
0.0027 - 0.0682 umol/mmol creatinine Adult (>18 years old) Both Normal
Urine Detected and Quantified
71.23 umol/mmol creatinine Adult (>18 years old) Male Normal
  • Shaykhutdino...
Urine Detected but not Quantified
Not Applicable Adult (>18 years old) Both Comment Normal
Urine Detected and Quantified
128.1 +/- 65.5 umol/mmol creatinine Adult (>18 years old) Male Normal
Urine Detected and Quantified
187.6 +/- 92.3 umol/mmol creatinine Adult (>18 years old) Female Normal
Urine Detected and Quantified
15.0 (10.0-23.0) umol/mmol creatinine Newborn (0-30 days old) Both Normal
  • Geigy Scient...
  • West Cadwell...
  • Basel, Switz...
Urine Detected and Quantified
30.0 +/- 19.0 umol/mmol creatinine Children (1-13 year old) Both Normal
  • Geigy Scient...
  • West Cadwell...
  • Basel, Switz...
Urine Detected but not Quantified
Not Applicable Adult (>18 years old) Male Comment Normal
Urine Detected and Quantified
92.0 +/- 39.0 umol/mmol creatinine Adult (>18 years old) Male Normal
  • Geigy Scient...
  • West Cadwell...
  • Basel, Switz...
Urine Detected but not Quantified
Not Applicable Adult (>18 years old) Male Comment Normal
Urine Detected and Quantified
139.0 +/- 86.0 umol/mmol creatinine Adult (>18 years old) Female Normal
  • Geigy Scient...
  • West Cadwell...
  • Basel, Switz...
Urine Detected and Quantified
136.48 +/- 77.27 umol/mmol creatinine Adult (>18 years old) Both Normal
Urine Detected and Quantified
101 (37.0-250.6) umol/mmol creatinine Adult (>18 years old) Both Comment Normal
Urine Detected and Quantified
118.9 umol/mmol creatinine Adult (>18 years old) Both Normal
Abnormal Concentrations
Biofluid Status Value Age Sex Condition Reference
Blood Detected and Quantified 234.9 +/- 181.1 uM Adult (>18 years old) Not Specified Heart Transplant
Blood Detected and Quantified 1090.0 (780.0-1400.0) uM Newborn (0-30 days old) Both Non-ketotic hyperglycinemia
Blood Detected and Quantified 1000.0 (800.0-1200.0) uM Children (1-13 year old) Both D-Glyceric acidura
Blood Detected and Quantified 210.0 (203.0-217.0) uM Adult (>18 years old) Both Refractory localization-related epilepsy (RLE)
Blood Detected and Quantified 354.0 (309.0-399.0) uM Children (1-13 year old) Both Juvenile myoclonic epilepsy (JME)
Blood Detected and Quantified 0.153 +/- 0.012 uM Elderly (>65 years old) Both Alzheimer's disease
Cerebrospinal Fluid (CSF) Detected and Quantified 9.7 +/- 4.9 uM Children (1-13 year old) Not Specified Leukemia
Cerebrospinal Fluid (CSF) Detected and Quantified 13.4 +/- 6.4 uM Children (1-13 year old) Not Specified Comment Leukemia
Cerebrospinal Fluid (CSF) Detected and Quantified 9.0405 (8.716-9.623) uM Not Specified Not Specified control
Cerebrospinal Fluid (CSF) Detected and Quantified 4.5 +/- 0.75 uM Adult (>18 years old) Both Schizophrenia
Cerebrospinal Fluid (CSF) Detected and Quantified 0.28 +/- 0.12 uM Adult (>18 years old) Both Alzheimer's disease
Urine Detected and Quantified 3000.0 (1000.0-5000.0) umol/mmol creatinine Children (1-13 year old) Both Carbamoyl phosphate synthetase deficiency
Urine Detected and Quantified 0.0024 - 0.0339 umol/mmol creatinine Adult (>18 years old) Both ADPKD
Urine Detected and Quantified 84.0 +/- 67.0 umol/mmol creatinine Adult (>18 years old) Both Lung cancer
Urine Detected and Quantified 0.010 +/- 0.001 umol/mmol creatinine Adult (>18 years old) Both Alzheimer's disease
Urine Detected and Quantified 178.2 +/- 6.4 umol/mmol creatinine Adult (>18 years old) Both 3-Hydroxy-3- methylglutaryl-CoA lyase (HL) deficency
Urine Detected and Quantified 135.8 +/- 5.8 umol/mmol creatinine Adult (>18 years old) Both 3-Hydroxy-3- methylglutaryl-CoA lyase (HL) deficency
Urine Detected and Quantified 53.1 +/- 1.8 umol/mmol creatinine Adult (>18 years old) Both Arginosuccinic aciduria
Urine Detected and Quantified 585.5 +/- 32.1 umol/mmol creatinine Adult (>18 years old) Both Propionic acidemia
Urine Detected and Quantified 550.00 (100.00-1000.00) umol/mmol creatinine Children (1-13 year old) Both Iminoglycinuria
Urine Detected and Quantified 2040.3 +/- 202.6 umol/mmol creatinine Adult (>18 years old) Both Propionic acidemia
Urine Detected and Quantified 49.9 +/- 1.3 umol/mmol creatinine Adult (>18 years old) Both Tyrosinemia I
Urine Detected and Quantified 176.2 +/- 9.2 umol/mmol creatinine Adult (>18 years old) Both Tyrosinemia I
Urine Detected and Quantified 82.2 +/- 4.1 umol/mmol creatinine Adult (>18 years old) Both Phenylketonuria
Urine Detected and Quantified 304.8 +/- 11.3 umol/mmol creatinine Adult (>18 years old) Both Phenylketonuria
Urine Detected and Quantified 233.7 +/- 10.5 umol/mmol creatinine Adult (>18 years old) Both Maple syrup urine disease
Urine Detected and Quantified 3205.1 +/- 83.4 umol/mmol creatinine Adult (>18 years old) Both Aminoaciduria
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 Link_out
    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 Link_out
        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 Link_out
            Lung Cancer
            • 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 Link_out
                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 Link_out
                    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 Link_out
                        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 Link_out
                            Carbamoyl Phosphate Synthetase Deficiency
                                Iminoglycinuria
                                    Associated OMIM IDs
                                    DrugBank ID DB00145 Link_out
                                    DrugBank Metabolite ID Not Available
                                    Phenol Explorer Compound ID Not Available
                                    Phenol Explorer Metabolite ID 1062 Link_out
                                    FoodDB ID FDB000484
                                    KNApSAcK ID C00001361 Link_out
                                    Chemspider ID 730 Link_out
                                    KEGG Compound ID C00037 Link_out
                                    BioCyc ID GLY Link_out
                                    BiGG ID 33610 Link_out
                                    Wikipedia Link Glycine Link_out
                                    NuGOwiki Link HMDB00123 Link_out
                                    Metagene Link HMDB00123 Link_out
                                    METLIN ID 20 Link_out
                                    PubChem Compound 750 Link_out
                                    PDB ID GLY Link_out
                                    ChEBI ID 15428 Link_out
                                    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. 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 Link_out
                                    2. 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 Link_out
                                    3. 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 Link_out
                                    4. 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 Link_out
                                    5. 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 Link_out
                                    6. 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 Link_out
                                    7. 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 Link_out
                                    8. 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 Link_out
                                    9. 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 Link_out
                                    10. 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 Link_out
                                    11. 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 Link_out
                                    12. 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 Link_out
                                    13. 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 Link_out
                                    14. 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 Link_out
                                    15. 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 Link_out
                                    16. 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 Link_out
                                    17. 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 Link_out
                                    18. 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 Link_out
                                    19. 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 Link_out
                                    20. 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 Link_out
                                    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 Link_out

                                    Enzymes
                                    Name: 5-aminolevulinate synthase, erythroid-specific, mitochondrial
                                    Reactions:
                                    Succinyl-CoA + Glycine unknown 5-Aminolevulinic acid + Coenzyme A + CO(2) details
                                    Succinyl-CoA + Glycine unknown 5-Aminolevulinic acid + Coenzyme A + Carbon dioxide details
                                    Gene Name: ALAS2
                                    Uniprot ID: P22557 Link_out
                                    Protein Sequence: FASTA
                                    Gene Sequence: FASTA
                                    Name: 5-aminolevulinate synthase, nonspecific, mitochondrial
                                    Reactions:
                                    Succinyl-CoA + Glycine unknown 5-Aminolevulinic acid + Coenzyme A + CO(2) details
                                    Succinyl-CoA + Glycine unknown 5-Aminolevulinic acid + Coenzyme A + Carbon dioxide details
                                    Gene Name: ALAS1
                                    Uniprot ID: P13196 Link_out
                                    Protein Sequence: FASTA
                                    Gene Sequence: FASTA
                                    Name: 2-amino-3-ketobutyrate coenzyme A ligase, mitochondrial
                                    Reactions:
                                    Acetyl-CoA + Glycine unknown Coenzyme A + L-2-Amino-3-oxobutanoic acid details
                                    Acetyl-CoA + Glycine unknown Coenzyme A + L-2-Amino-3-oxobutanoic acid details
                                    Gene Name: GCAT
                                    Uniprot ID: O75600 Link_out
                                    Protein Sequence: FASTA
                                    Gene Sequence: FASTA
                                    Name: Peroxisomal sarcosine oxidase
                                    Reactions:
                                    Sarcosine + Water + Oxygen unknown Glycine + Formaldehyde + Hydrogen peroxide details
                                    Gene Name: PIPOX
                                    Uniprot ID: Q9P0Z9 Link_out
                                    Protein Sequence: FASTA
                                    Gene Sequence: FASTA
                                    Name: D-amino-acid oxidase
                                    Reactions:
                                    Glycine + Water + Oxygen unknown Glyoxylic acid + Ammonia + Hydrogen peroxide details
                                    Gene Name: DAO
                                    Uniprot ID: P14920 Link_out
                                    Protein Sequence: FASTA
                                    Gene Sequence: FASTA
                                    Name: Trifunctional purine biosynthetic protein adenosine-3
                                    Reactions:
                                    Adenosine triphosphate + 5-Phosphoribosylamine + Glycine unknown ADP + Phosphoric acid + Glycineamideribotide details
                                    Adenosine triphosphate + 5-Phosphoribosylamine + Glycine unknown ADP + Phosphoric acid + Glycineamideribotide details
                                    Gene Name: GART
                                    Uniprot ID: P22102 Link_out
                                    Protein Sequence: FASTA
                                    Gene Sequence: FASTA
                                    Name: Alanine--glyoxylate aminotransferase 2, mitochondrial
                                    Reactions:
                                    L-Alanine + Glyoxylic acid unknown Pyruvic acid + Glycine details
                                    Glycine + Oxoglutaric acid unknown Glyoxylic acid + L-Glutamic acid details
                                    Gene Name: AGXT2
                                    Uniprot ID: Q9BYV1 Link_out
                                    Protein Sequence: FASTA
                                    Gene Sequence: FASTA
                                    Name: Glycine N-methyltransferase
                                    Reactions:
                                    S-Adenosylmethionine + Glycine unknown S-Adenosylhomocysteine + Sarcosine details
                                    Gene Name: GNMT
                                    Uniprot ID: Q14749 Link_out
                                    Protein Sequence: FASTA
                                    Gene Sequence: FASTA
                                    Name: Aminopeptidase N
                                    Reactions:
                                    Cysteinylglycine + Water unknown L-Cysteine + Glycine details
                                    R-S-Cysteinylglycine + Water unknown S-Substituted L-cysteine + Glycine details
                                    Gene Name: ANPEP
                                    Uniprot ID: P15144 Link_out
                                    Protein Sequence: FASTA
                                    Gene Sequence: FASTA
                                    Name: Glycine amidinotransferase, mitochondrial
                                    Reactions:
                                    L-Arginine + Glycine unknown Ornithine + Guanidoacetic acid details
                                    Gene Name: GATM
                                    Uniprot ID: P50440 Link_out
                                    Protein Sequence: FASTA
                                    Gene Sequence: FASTA
                                    Name: Bile acid-CoA:amino acid N-acyltransferase
                                    Reactions:
                                    Choloyl-CoA + Glycine unknown Coenzyme A + Glycocholic acid details
                                    Chenodeoxycholoyl-CoA + Glycine unknown Chenodeoxycholic acid glycine conjugate + Coenzyme A details
                                    Gene Name: BAAT
                                    Uniprot ID: Q14032 Link_out
                                    Protein Sequence: FASTA
                                    Gene Sequence: FASTA
                                    Name: Glycine--tRNA ligase
                                    Reactions:
                                    Adenosine triphosphate + Glycine + tRNA(Gly) unknown Adenosine monophosphate + Pyrophosphate + glycyl-tRNA(Gly) details
                                    Adenosine triphosphate + Glycine + tRNA(Gly) unknown Adenosine monophosphate + Pyrophosphate + Glycyl-tRNA(Gly) details
                                    Gene Name: GARS
                                    Uniprot ID: P41250 Link_out
                                    Protein Sequence: FASTA
                                    Gene Sequence: FASTA
                                    Name: Aminomethyltransferase, mitochondrial
                                    Reactions:
                                    Glycine + Tetrahydrofolic acid + NAD unknown 5,10-Methylene-THF + Ammonia + Carbon dioxide + NADH + Hydrogen Ion details
                                    Gene Name: AMT
                                    Uniprot ID: P48728 Link_out
                                    Protein Sequence: FASTA
                                    Gene Sequence: FASTA
                                    Name: Serine--pyruvate aminotransferase
                                    Reactions:
                                    L-Alanine + Glyoxylic acid unknown Pyruvic acid + Glycine details
                                    Glycine + Oxoglutaric acid unknown Glyoxylic acid + L-Glutamic acid details
                                    L-Serine + Glyoxylic acid unknown Hydroxypyruvic acid + Glycine details
                                    Gene Name: AGXT
                                    Uniprot ID: P21549 Link_out
                                    Protein Sequence: FASTA
                                    Gene Sequence: FASTA
                                    Name: Glutathione synthetase
                                    Reactions:
                                    Adenosine triphosphate + Gamma-Glutamylcysteine + Glycine unknown ADP + Phosphoric acid + Glutathione details
                                    Gene Name: GSS
                                    Uniprot ID: P48637 Link_out
                                    Protein Sequence: FASTA
                                    Gene Sequence: FASTA
                                    Name: Serine hydroxymethyltransferase, mitochondrial
                                    Reactions:
                                    5,10-Methylene-THF + Glycine + Water unknown Tetrahydrofolic acid + L-Serine details
                                    L-Serine + 5,6,7,8-Tetrahydromethanopterin unknown 5,10-Methylenetetrahydromethanopterin + Glycine + Water details
                                    Gene Name: SHMT2
                                    Uniprot ID: P34897 Link_out
                                    Protein Sequence: FASTA
                                    Gene Sequence: FASTA
                                    Name: Sarcosine dehydrogenase, mitochondrial
                                    Reactions:
                                    Sarcosine + Water + electron-transfer flavoprotein unknown Glycine + Formaldehyde + reduced electron-transfer flavoprotein details
                                    Sarcosine + Electron-transferring flavoprotein + Water unknown Glycine + Formaldehyde + Reduced electron-transferring flavoprotein details
                                    Gene Name: SARDH
                                    Uniprot ID: Q9UL12 Link_out
                                    Protein Sequence: FASTA
                                    Gene Sequence: FASTA
                                    Name: Serine hydroxymethyltransferase, cytosolic
                                    Reactions:
                                    5,10-Methylene-THF + Glycine + Water unknown Tetrahydrofolic acid + L-Serine details
                                    L-Serine + 5,6,7,8-Tetrahydromethanopterin unknown 5,10-Methylenetetrahydromethanopterin + Glycine + Water details
                                    Gene Name: SHMT1
                                    Uniprot ID: P34896 Link_out
                                    Protein Sequence: FASTA
                                    Gene Sequence: FASTA
                                    Name: Glycine dehydrogenase [decarboxylating], mitochondrial
                                    Reactions:
                                    Glycine + H-protein-lipoyllysine unknown H-protein-S-aminomethyldihydrolipoyllysine + CO(2) details
                                    Glycine + Lipoylprotein unknown S-Aminomethyldihydrolipoylprotein + Carbon dioxide details
                                    Gene Name: GLDC
                                    Uniprot ID: P23378 Link_out
                                    Protein Sequence: FASTA
                                    Gene Sequence: FASTA
                                    Name: Cytosol aminopeptidase
                                    Reactions:
                                    Cysteinylglycine + Water unknown L-Cysteine + Glycine details
                                    R-S-Cysteinylglycine + Water unknown S-Substituted L-cysteine + Glycine details
                                    Gene Name: LAP3
                                    Uniprot ID: P28838 Link_out
                                    Protein Sequence: FASTA
                                    Gene Sequence: FASTA
                                    Name: Glutamate [NMDA] receptor subunit 3B
                                    Reactions: Not Available
                                    Gene Name: GRIN3B
                                    Uniprot ID: O60391 Link_out
                                    Protein Sequence: FASTA
                                    Gene Sequence: FASTA
                                    Name: Glutamate [NMDA] receptor subunit epsilon-1
                                    Reactions: Not Available
                                    Gene Name: GRIN2A
                                    Uniprot ID: Q12879 Link_out
                                    Protein Sequence: FASTA
                                    Gene Sequence: FASTA
                                    Name: Glutamate [NMDA] receptor subunit epsilon-3
                                    Reactions: Not Available
                                    Gene Name: GRIN2C
                                    Uniprot ID: Q14957 Link_out
                                    Protein Sequence: FASTA
                                    Gene Sequence: FASTA
                                    Name: Sodium- and chloride-dependent glycine transporter 1
                                    Reactions: Not Available
                                    Gene Name: SLC6A9
                                    Uniprot ID: P48067 Link_out
                                    Protein Sequence: FASTA
                                    Gene Sequence: FASTA
                                    Name: Glycine cleavage system H protein, mitochondrial
                                    Reactions:
                                    Glycine + Tetrahydrofolic acid + NAD unknown 5,10-Methylene-THF + Ammonia + Carbon dioxide + NADH + Hydrogen Ion details
                                    Gene Name: GCSH
                                    Uniprot ID: P23434 Link_out
                                    Protein Sequence: FASTA
                                    Gene Sequence: FASTA
                                    Name: Sodium- and chloride-dependent glycine transporter 2
                                    Reactions: Not Available
                                    Gene Name: SLC6A5
                                    Uniprot ID: Q9Y345 Link_out
                                    Protein Sequence: FASTA
                                    Gene Sequence: FASTA
                                    Name: Vesicular inhibitory amino acid transporter
                                    Reactions: Not Available
                                    Gene Name: SLC32A1
                                    Uniprot ID: Q9H598 Link_out
                                    Protein Sequence: FASTA
                                    Gene Sequence: FASTA
                                    Name: Glycine N-acyltransferase
                                    Reactions:
                                    Acyl-CoA + Glycine unknown Coenzyme A + N-acylglycine details
                                    Benzoyl-CoA + Glycine unknown Coenzyme A + Hippuric acid details
                                    Phenylacetyl-CoA + Glycine unknown Phenylacetylglycine + Coenzyme A details
                                    Gene Name: GLYAT
                                    Uniprot ID: Q6IB77 Link_out
                                    Protein Sequence: FASTA
                                    Gene Sequence: FASTA
                                    Name: Glycine N-acyltransferase-like protein 1
                                    Reactions: Not Available
                                    Gene Name: GLYATL1
                                    Uniprot ID: Q969I3 Link_out
                                    Protein Sequence: FASTA
                                    Gene Sequence: FASTA
                                    Name: Glycine N-acyltransferase-like protein 2
                                    Reactions:
                                    Acyl-CoA + Glycine unknown Coenzyme A + N-acylglycine details
                                    Gene Name: GLYATL2
                                    Uniprot ID: Q8WU03 Link_out
                                    Protein Sequence: FASTA
                                    Gene Sequence: FASTA
                                    Name: Glycine receptor subunit alpha-1
                                    Reactions: Not Available
                                    Gene Name: GLRA1
                                    Uniprot ID: P23415 Link_out
                                    Protein Sequence: FASTA
                                    Gene Sequence: FASTA
                                    Name: Glycine receptor subunit alpha-2
                                    Reactions: Not Available
                                    Gene Name: GLRA2
                                    Uniprot ID: P23416 Link_out
                                    Protein Sequence: FASTA
                                    Gene Sequence: FASTA
                                    Name: Glycine receptor subunit alpha-3
                                    Reactions: Not Available
                                    Gene Name: GLRA3
                                    Uniprot ID: O75311 Link_out
                                    Protein Sequence: FASTA
                                    Gene Sequence: FASTA
                                    Name: Glycine receptor subunit beta
                                    Reactions: Not Available
                                    Gene Name: GLRB
                                    Uniprot ID: P48167 Link_out
                                    Protein Sequence: FASTA
                                    Gene Sequence: FASTA
                                    Name: Kynurenine--oxoglutarate transaminase 3
                                    Reactions:
                                    L-Kynurenine + Glyoxylic acid unknown 4-(2-Aminophenyl)-2,4-dioxobutanoic acid + Glycine details
                                    Gene Name: CCBL2
                                    Uniprot ID: Q6YP21 Link_out
                                    Protein Sequence: FASTA
                                    Gene Sequence: FASTA
                                    Name: Serine hydroxymethyltransferase
                                    Reactions: Not Available
                                    Gene Name: Not Available
                                    Uniprot ID: Q53ET4 Link_out
                                    Protein Sequence: FASTA
                                    Gene Sequence: FASTA
                                    Name: Serine hydroxymethyltransferase
                                    Reactions: Not Available
                                    Gene Name: SHMT2
                                    Uniprot ID: Q5BJF5 Link_out
                                    Protein Sequence: FASTA
                                    Gene Sequence: FASTA
                                    Name: Serine hydroxymethyltransferase
                                    Reactions: Not Available
                                    Gene Name: DKFZp686P09201
                                    Uniprot ID: Q5HYG8 Link_out
                                    Protein Sequence: FASTA
                                    Gene Sequence: FASTA
                                    Name: N-arachidonyl glycine receptor
                                    Reactions: Not Available
                                    Gene Name: GPR18
                                    Uniprot ID: Q14330 Link_out
                                    Protein Sequence: FASTA
                                    Gene Sequence: FASTA
                                    Transporters
                                    Name: Proton-coupled amino acid transporter 1
                                    Reactions: Not Available
                                    Gene Name: SLC36A1
                                    Uniprot ID: Q7Z2H8 Link_out
                                    Protein Sequence: FASTA
                                    Gene Sequence: FASTA
                                    Name: Monocarboxylate transporter 10
                                    Reactions: Not Available
                                    Gene Name: SLC16A10
                                    Uniprot ID: Q8TF71 Link_out
                                    Protein Sequence: FASTA
                                    Gene Sequence: FASTA