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Record Information
Creation Date2005-11-16 15:48:42 UTC
Update Date2017-07-18 21:45:33 UTC
Secondary Accession NumbersNone
Metabolite Identification
Common NameGuanidoacetic acid
DescriptionGuanidoacetic acid is a metabolite in the Urea cycle and metabolism of amino groups, and in the metabolic pathways of several amino acids. This includes glycine, serine, threonine, arginine and proline metabolism. Guanidinoacetic acid is also a precursor of creatine, an essential substrate for muscle energy metabolism.
Guanidinoacetic acidGenerator
2-[[amino(imino)Methyl]amino]acetic acidHMDB
a-Guanidinoacetic acidHMDB
alpha-Guanidinoacetic acidHMDB
b-Guanidinoacetic acidHMDB
beta-Guanidinoacetic acidHMDB
Guanidineacetic acidHMDB
Guanidylacetic acidHMDB
Guanyl glycineHMDB
[(Aminoiminomethyl)amino]-acetic acidHMDB
Glycocyamine, 2-(14)C-labeledMeSH
Glycocyamine, ion (1-)MeSH
Glycocyamine monohydrochlorideMeSH
Chemical FormulaC3H7N3O2
Average Molecular Weight117.1066
Monoisotopic Molecular Weight117.053826483
IUPAC Name2-carbamimidamidoacetic acid
Traditional Nameglycocyamine
CAS Registry Number352-97-6
InChI Identifier
Chemical Taxonomy
DescriptionThis compound belongs to the class of organic compounds known as alpha amino acids and derivatives. These are amino acids in which the amino group is attached to the carbon atom immediately adjacent to the carboxylate group (alpha carbon), or a derivative thereof.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassCarboxylic acids and derivatives
Sub ClassAmino acids, peptides, and analogues
Direct ParentAlpha amino acids and derivatives
Alternative Parents
  • Alpha-amino acid or derivatives
  • Guanidine
  • Carboximidamide
  • Monocarboxylic acid or derivatives
  • Carboxylic acid
  • Organic nitrogen compound
  • Organic oxygen compound
  • Organopnictogen compound
  • Organic oxide
  • Hydrocarbon derivative
  • Organooxygen compound
  • Organonitrogen compound
  • Imine
  • Carbonyl group
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
StatusDetected and Quantified
  • Endogenous
  • Component of Arginine and proline metabolism
  • Component of Glycine, serine and threonine metabolism
ApplicationNot Available
Cellular locations
  • Cytoplasm (predicted from logP)
Physical Properties
Experimental Properties
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water Solubility3.6 mg/mL at 15 °CNot Available
LogPNot AvailableNot Available
Predicted Properties
Water Solubility4.19 mg/mLALOGPS
pKa (Strongest Acidic)3.37ChemAxon
pKa (Strongest Basic)12.24ChemAxon
Physiological Charge0ChemAxon
Hydrogen Acceptor Count5ChemAxon
Hydrogen Donor Count4ChemAxon
Polar Surface Area99.2 Å2ChemAxon
Rotatable Bond Count2ChemAxon
Refractivity36.72 m3·mol-1ChemAxon
Polarizability10.45 Å3ChemAxon
Number of Rings0ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectrum TypeDescriptionSplash Key
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-016r-7900000000-4777d20e1ab29262427eView in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-00dl-9000000000-a1b5501bf0f7a9b0489dView in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)splash10-0006-9100000000-b61a48cf749b57a2c77eView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) , Positivesplash10-0gk9-7900000000-5ba298286886c1fabdf5View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) , Negativesplash10-00di-9100000000-a5b77a913821d637210fView in MoNA
LC-MS/MSLC-MS/MS Spectrum - , positivesplash10-014i-6900000000-4d655a894c702bdfdb59View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, PositiveNot Available
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, PositiveNot Available
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, PositiveNot Available
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, NegativeNot Available
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, NegativeNot Available
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, NegativeNot Available
1D NMR1H NMR SpectrumNot Available
1D NMR13C NMR SpectrumNot Available
2D NMR[1H,13C] 2D NMR SpectrumNot Available
Biological Properties
Cellular Locations
  • Cytoplasm (predicted from logP)
Biofluid Locations
  • Blood
  • Cellular Cytoplasm
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Urine
Tissue Location
  • Brain
  • Kidney
  • Liver
3-Phosphoglycerate dehydrogenase deficiencySMP00721Not Available
Arginine and Proline MetabolismSMP00020map00330
Arginine: Glycine Amidinotransferase Deficiency (AGAT Deficiency)SMP00362Not Available
Creatine deficiency, guanidinoacetate methyltransferase deficiencySMP00504Not Available
Dihydropyrimidine Dehydrogenase Deficiency (DHPD)SMP00179Not Available
Dimethylglycine Dehydrogenase DeficiencySMP00242Not Available
Dimethylglycine Dehydrogenase DeficiencySMP00484Not Available
Glycine and Serine MetabolismSMP00004map00260
Guanidinoacetate Methyltransferase Deficiency (GAMT Deficiency)SMP00188Not Available
Hyperglycinemia, non-ketoticSMP00485Not Available
Hyperornithinemia with gyrate atrophy (HOGA)SMP00505Not Available
Hyperornithinemia-hyperammonemia-homocitrullinuria [HHH-syndrome]SMP00506Not Available
Hyperprolinemia Type ISMP00361Not Available
Hyperprolinemia Type IISMP00360Not Available
L-arginine:glycine amidinotransferase deficiencySMP00507Not Available
Non Ketotic HyperglycinemiaSMP00223Not Available
Ornithine Aminotransferase Deficiency (OAT Deficiency)SMP00363Not Available
Prolidase Deficiency (PD)SMP00207Not Available
Prolinemia Type IISMP00208Not Available
SarcosinemiaSMP00244Not Available
Normal Concentrations
BloodDetected and Quantified2.8 +/- 0.9 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified1.90 +/- 0.68 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified3.08 (0.87-3.64) uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified19.0 (3.0-37.0) uMAdult (>18 years old)Not SpecifiedNormal
    • Geigy Scientific ...
BloodDetected and Quantified2.0 (0.0 - 5.0) uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified16.8 (0.81-32.9) uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified1.22 +/- 0.40 uMAdult (>18 years old)BothNormal details
Cellular CytoplasmDetected and Quantified2.3 +/- 0.9 uMAdult (>18 years old)Not SpecifiedNormal
    • Geigy Scientific ...
Cerebrospinal Fluid (CSF)Detected and Quantified0.05 +/- 0.03 uMAdult (>18 years old)BothNormal details
FecesDetected but not Quantified Children (6 - 18 years old)Not SpecifiedNormal details
UrineDetected but not Quantified Adult (>18 years old)BothNormal details
UrineDetected and Quantified16-228 umol/mmol creatinineChildren (1 - 18 years old)Both
    • BC Children's Hos...
UrineDetected and Quantified12.81-38.44 umol/mmol creatinineAdult (>18 years old)BothNormal
    • David F. Putnam C...
UrineDetected and Quantified7.69-21.36 umol/mmol creatinineAdult (>18 years old)BothNormal
    • David F. Putnam C...
UrineDetected and Quantified15.4 +/- 10.0 umol/mmol creatinineAdult (>18 years old)BothNormal
    • Geigy Scientific ...
    • West Cadwell, N.J...
    • Basel, Switzerlan...
UrineDetected and Quantified41.8 (10.6-97.3) umol/mmol creatinineAdult (>18 years old)Both
UrineDetected and Quantified9.33 umol/mmol creatinineAdult (>18 years old)MaleNormal
    • Shaykhutdinov RA,...
UrineDetected and Quantified89.0 (11.0-124.0) umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified88.5 (18.0-159.0) umol/mmol creatinineInfant (0-1 year old)BothNormal details
UrineDetected and Quantified74.0 (18.0-130.0) umol/mmol creatinineChildren (1-13 years old)BothNormal details
UrineDetected and Quantified13.3 (5.2-21.5) umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected but not Quantified Adult (>18 years old)Both
UrineDetected and Quantified115.283 +/- 130.147 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
    • Mordechai, Hien, ...
Abnormal Concentrations
BloodDetected and Quantified2.4 +/- 0.7 uMAdult (>18 years old)BothChronic renal failure details
BloodDetected and Quantified1.88 +/- 0.30 uMAdult (>18 years old)Bothuremia details
FecesDetected but not Quantified Children (6 - 18 years old)Not SpecifiedCrohns disease details
FecesDetected but not Quantified Children (6 - 18 years old)Not SpecifiedUlcerative colitis details
FecesDetected but not Quantified Children (6 - 18 years old)Not SpecifiedUnclassified IBD details
UrineDetected but not Quantified Adult (>18 years old)Both
UrineDetected and Quantified55.592 +/- 42.373 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Eosinophilic esophagitis
    • Mordechai, Hien, ...
Associated Disorders and Diseases
Disease References
Chronic renal failure
  1. Kikuchi T, Orita Y, Ando A, Mikami H, Fujii M, Okada A, Abe H: Liquid-chromatographic determination of guanidino compounds in plasma and erythrocyte of normal persons and uremic patients. Clin Chem. 1981 Nov;27(11):1899-902. [PubMed:7296840 ]
  1. Yang J, Chen T, Sun L, Zhao Z, Qi X, Zhou K, Cao Y, Wang X, Qiu Y, Su M, Zhao A, Wang P, Yang P, Wu J, Feng G, He L, Jia W, Wan C: Potential metabolite markers of schizophrenia. Mol Psychiatry. 2013 Jan;18(1):67-78. doi: 10.1038/mp.2011.131. Epub 2011 Oct 25. [PubMed:22024767 ]
Associated OMIM IDs
DrugBank IDNot Available
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB021898
KNApSAcK IDNot Available
Chemspider ID743
KEGG Compound IDC00581
BiGG ID1594816
Wikipedia LinkNot Available
NuGOwiki LinkHMDB00128
Metagene LinkHMDB00128
PubChem Compound763
ChEBI ID16344
Synthesis ReferenceThalhammer, Franz; Gastner, Thomas. Preparation of creatine, creatine monohydrate and guanidinoacetic acid. Ger. Offen. (2007), 5pp.
Material Safety Data Sheet (MSDS)Not Available
General References
  1. Isbrandt D, von Figura K: Cloning and sequence analysis of human guanidinoacetate N-methyltransferase cDNA. Biochim Biophys Acta. 1995 Dec 27;1264(3):265-7. [PubMed:8547310 ]
  2. Stockler S, Isbrandt D, Hanefeld F, Schmidt B, von Figura K: Guanidinoacetate methyltransferase deficiency: the first inborn error of creatine metabolism in man. Am J Hum Genet. 1996 May;58(5):914-22. [PubMed:8651275 ]
  3. Mercimek-Mahmutoglu S, Stoeckler-Ipsiroglu S, Adami A, Appleton R, Araujo HC, Duran M, Ensenauer R, Fernandez-Alvarez E, Garcia P, Grolik C, Item CB, Leuzzi V, Marquardt I, Muhl A, Saelke-Kellermann RA, Salomons GS, Schulze A, Surtees R, van der Knaap MS, Vasconcelos R, Verhoeven NM, Vilarinho L, Wilichowski E, Jakobs C: GAMT deficiency: features, treatment, and outcome in an inborn error of creatine synthesis. Neurology. 2006 Aug 8;67(3):480-4. Epub 2006 Jul 19. [PubMed:16855203 ]
  4. Schulze A: Creatine deficiency syndromes. Mol Cell Biochem. 2003 Feb;244(1-2):143-50. [PubMed:12701824 ]
  5. Ensenauer R, Thiel T, Schwab KO, Tacke U, Stockler-Ipsiroglu S, Schulze A, Hennig J, Lehnert W: Guanidinoacetate methyltransferase deficiency: differences of creatine uptake in human brain and muscle. Mol Genet Metab. 2004 Jul;82(3):208-13. [PubMed:15234333 ]
  6. Yasuda M, Sugahara K, Zhang J, Ageta T, Nakayama K, Shuin T, Kodama H: Simultaneous determination of creatinine, creatine, and guanidinoacetic acid in human serum and urine using liquid chromatography-atmospheric pressure chemical ionization mass spectrometry. Anal Biochem. 1997 Nov 15;253(2):231-5. [PubMed:9367508 ]
  7. Sijens PE, Reijngoud DJ, Soorani-Lunsing RJ, Oudkerk M, van Spronsen FJ: Cerebral 1H MR spectroscopy showing elevation of brain guanidinoacetate in argininosuccinate lyase deficiency. Mol Genet Metab. 2006 May;88(1):100-2. Epub 2005 Dec 15. [PubMed:16343968 ]
  8. Valongo C, Cardoso ML, Domingues P, Almeida L, Verhoeven N, Salomons G, Jakobs C, Vilarinho L: Age related reference values for urine creatine and guanidinoacetic acid concentration in children and adolescents by gas chromatography-mass spectrometry. Clin Chim Acta. 2004 Oct;348(1-2):155-61. [PubMed:15369749 ]
  9. Fingerhut R: Stable isotope dilution method for the determination of guanidinoacetic acid by gas chromatography/mass spectrometry. Rapid Commun Mass Spectrom. 2003;17(7):717-22. [PubMed:12661026 ]
  10. Schmidt A, Marescau B, Boehm EA, Renema WK, Peco R, Das A, Steinfeld R, Chan S, Wallis J, Davidoff M, Ullrich K, Waldschutz R, Heerschap A, De Deyn PP, Neubauer S, Isbrandt D: Severely altered guanidino compound levels, disturbed body weight homeostasis and impaired fertility in a mouse model of guanidinoacetate N-methyltransferase (GAMT) deficiency. Hum Mol Genet. 2004 May 1;13(9):905-21. Epub 2004 Mar 17. [PubMed:15028668 ]
  11. Shirokane Y, Utsushikawa M, Nakajima M: A new enzymic determination of guanidinoacetic acid in urine. Clin Chem. 1987 Mar;33(3):394-7. [PubMed:3815805 ]
  12. Mizutani N, Hayakawa C, Ohya Y, Watanabe K, Watanabe Y, Mori A: Guanidino compounds in hyperargininemia. Tohoku J Exp Med. 1987 Nov;153(3):197-205. [PubMed:3433275 ]
  13. Schulze A, Ebinger F, Rating D, Mayatepek E: Improving treatment of guanidinoacetate methyltransferase deficiency: reduction of guanidinoacetic acid in body fluids by arginine restriction and ornithine supplementation. Mol Genet Metab. 2001 Dec;74(4):413-9. [PubMed:11749046 ]
  14. Stockler S, Holzbach U, Hanefeld F, Marquardt I, Helms G, Requart M, Hanicke W, Frahm J: Creatine deficiency in the brain: a new, treatable inborn error of metabolism. Pediatr Res. 1994 Sep;36(3):409-13. [PubMed:7808840 ]


General function:
Involved in guanidinoacetate N-methyltransferase activity
Specific function:
Not Available
Gene Name:
Uniprot ID:
Molecular weight:
S-Adenosylmethionine + Guanidoacetic acid → S-Adenosylhomocysteine + Creatinedetails
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:
Uniprot ID:
Molecular weight:
L-Arginine + Glycine → Ornithine + Guanidoacetic aciddetails