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Record Information
Version4.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2019-07-25 18:11:43 UTC
HMDB IDHMDB0000128
Secondary Accession Numbers
  • HMDB00128
Metabolite Identification
Common NameGuanidoacetic acid
DescriptionGuanidoacetic acid, also known as guanidinoacetate or N-amidinoglycine, 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. Guanidinoacetic acid was first prepared in 1861 by Adolph Strecker by reaction of cyanamide with glycine in aqueous solution:Glycine can also be converted to glycocyamine with S-methylisothiourea or with O-alkylisoureas as a guanylation agent. Guanidinoacetic acid is a feed additive approved by EFSA in poultry farming (trade name: creAMINO). The simultaneous intake of methyl providing substances such as betaine appears advisable because of the risk of homocysteine formation with glycocyamine alone. Glycocyamine is formed in the mammalian organism primarily in the kidneys by transferring the guanidine group of L-arginine by the enzyme L-Arg:Gly-amidinotransferase (AGAT) to the amino acid glycine. Guanidoacetic acid is a drug. Guanidoacetic acid is a very strong basic compound (based on its pKa). Guanidoacetic acid exists in all living organisms, ranging from bacteria to humans. Within humans, guanidoacetic acid participates in a number of enzymatic reactions. In particular, ornithine and guanidoacetic acid can be biosynthesized from L-arginine and glycine through its interaction with the enzyme glycine amidinotransferase, mitochondrial. In addition, S-adenosylmethionine and guanidoacetic acid can be converted into S-adenosylhomocysteine and creatine through the action of the enzyme guanidinoacetate N-methyltransferase. A series of studies showed that a combination of betaine and glycocyamine improves the symptoms of patients with chronic illness, including heart disease, without toxicity. In humans, guanidoacetic acid is involved in the metabolic disorder called prolinemia type II. Guanidoacetic acid is a potentially toxic compound.
Structure
Data?1563860634
Synonyms
ValueSource
GlycocyamineChEBI
GuanidinoacetateChEBI
N-(Carbamimidoyl)glycineChEBI
N-[Amino(imino)methyl]glycineChEBI
N-AmidinoglycineChEBI
Guanidinoacetic acidKegg
GuanidoacetateGenerator
(Carboxymethyl)-guanidineHMDB
2-[[Amino(imino)methyl]amino]acetateHMDB
2-[[Amino(imino)methyl]amino]acetic acidHMDB
a-GuanidinoacetateHMDB
a-Guanidinoacetic acidHMDB
alpha-GuanidinoacetateHMDB
alpha-Guanidinoacetic acidHMDB
b-GuanidinoacetateHMDB
b-Guanidinoacetic acidHMDB
beta-GuanidinoacetateHMDB
beta-Guanidinoacetic acidHMDB
BetacyamineHMDB
BetasyamineHMDB
GuanidineacetateHMDB
Guanidineacetic acidHMDB
GuanidylacetateHMDB
Guanidylacetic acidHMDB
Guanyl glycineHMDB
N-Amidino-glycineHMDB
[(Aminoiminomethyl)amino]-acetateHMDB
[(Aminoiminomethyl)amino]-acetic acidHMDB
Glycocyamine, 2-(14)C-labeledHMDB
Glycocyamine, ion (1-)HMDB
Glycocyamine monohydrochlorideHMDB
Chemical FormulaC3H7N3O2
Average Molecular Weight117.1066
Monoisotopic Molecular Weight117.053826483
IUPAC Name2-carbamimidamidoacetic acid
Traditional Nameglycocyamine
CAS Registry Number352-97-6
SMILES
NC(=N)NCC(O)=O
InChI Identifier
InChI=1S/C3H7N3O2/c4-3(5)6-1-2(7)8/h1H2,(H,7,8)(H4,4,5,6)
InChI KeyBPMFZUMJYQTVII-UHFFFAOYSA-N
Chemical Taxonomy
Description 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
Substituents
  • 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
Ontology
Physiological effect

Health effect:

Disposition

Route of exposure:

Source:

Biological location:

Process

Naturally occurring process:

Role

Biological role:

Industrial application:

Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water Solubility3.6 mg/mL at 15 °CNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility4.19 g/LALOGPS
logP-1.8ALOGPS
logP-3.1ChemAxon
logS-1.4ALOGPS
pKa (Strongest Acidic)3.37ChemAxon
pKa (Strongest Basic)12.24ChemAxon
Physiological Charge0ChemAxon
Hydrogen Acceptor Count5ChemAxon
Hydrogen Donor Count4ChemAxon
Polar Surface Area99.2 ŲChemAxon
Rotatable Bond Count2ChemAxon
Refractivity36.72 m³·mol⁻¹ChemAxon
Polarizability10.54 ųChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectrum TypeDescriptionSplash KeyView
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-00di-0901000000-4a89fe463d29379e6339JSpectraViewer | MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-00di-0911000000-542baca9dcca73225bf0JSpectraViewer | MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-006t-0911200000-bb7ea068f193f39c283bJSpectraViewer | MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-00di-0901000000-4a89fe463d29379e6339JSpectraViewer | MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-00di-0911000000-542baca9dcca73225bf0JSpectraViewer | MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-006t-0911200000-bb7ea068f193f39c283bJSpectraViewer | MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-0006-9100000000-c703c631a81e400681bdJSpectraViewer
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (1 TMS) - 70eV, Positivesplash10-00dl-9700000000-1dae820000c4f9ec1662JSpectraViewer
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-016r-7900000000-4777d20e1ab29262427eJSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-00dl-9000000000-a1b5501bf0f7a9b0489dJSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)splash10-0006-9100000000-b61a48cf749b57a2c77eJSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) , Positivesplash10-0gk9-7900000000-5ba298286886c1fabdf5JSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) , Negativesplash10-00di-9100000000-a5b77a913821d637210fJSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QTOF , negativesplash10-00di-9100000000-a5b77a913821d637210fJSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QTOF , positivesplash10-0gk9-7900000000-5ba298286886c1fabdf5JSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - , positivesplash10-014i-6900000000-4d655a894c702bdfdb59JSpectraViewer | MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-014i-4900000000-1b831140a3071dd98e0cJSpectraViewer
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-00di-9300000000-b27aea734be08f5d2ddcJSpectraViewer
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-00dl-9000000000-dea2f28faa5430d7e56fJSpectraViewer
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-00xr-9500000000-1ba345c7064e9cb70f9cJSpectraViewer
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-00di-9000000000-6864d4355d3462fbc69fJSpectraViewer
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-0006-9000000000-1c36299ab8746bbf67a5JSpectraViewer
1D NMR1H NMR SpectrumNot AvailableJSpectraViewer
1D NMR13C NMR SpectrumNot AvailableJSpectraViewer
2D NMR[1H,13C] 2D NMR SpectrumNot AvailableJSpectraViewer
Biological Properties
Cellular Locations
  • Cytoplasm (predicted from logP)
Biospecimen Locations
  • Blood
  • Cellular Cytoplasm
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Urine
Tissue Locations
  • Brain
  • Kidney
  • Liver
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified2.8 +/- 0.9 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified1.0-3.5 uMChildren (1-13 years old)Not SpecifiedNormal details
BloodDetected and Quantified0.35-8.0 uMChildren (1-13 years old)Not SpecifiedNormal details
BloodDetected and Quantified0.7-1.4 uMChildren (1-13 years old)BothNormal details
BloodDetected and Quantified0.7-3.5 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified1.3-3.3 uMAdult (>18 years old)FemaleNormal 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 ...
details
BloodDetected and Quantified1.900 +/- 0.680 uMAdult (>18 years old)Both
Normal
details
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 ...
details
Cerebrospinal Fluid (CSF)Detected and Quantified0.068-0.114 uMAdult (>18 years old)FemaleNormal details
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
FecesDetected but not Quantified Adult (>18 years old)Both
Normal
details
FecesDetected but not Quantified Adult (>18 years old)Both
Normal
details
UrineDetected but not Quantified Adult (>18 years old)BothNormal details
UrineDetected and Quantified16-228 umol/mmol creatinineChildren (1 - 18 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified12.81-38.44 umol/mmol creatinineAdult (>18 years old)BothNormal
    • David F. Putnam C...
details
UrineDetected and Quantified7.69-21.36 umol/mmol creatinineAdult (>18 years old)BothNormal
    • David F. Putnam C...
details
UrineDetected and Quantified15.4 +/- 10.0 umol/mmol creatinineAdult (>18 years old)BothNormal
    • Geigy Scientific ...
    • West Cadwell, N.J...
    • Basel, Switzerlan...
details
UrineDetected and Quantified41.8 (10.6-97.3) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified9.33 umol/mmol creatinineAdult (>18 years old)MaleNormal
    • Shaykhutdinov RA,...
details
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
Normal
details
UrineDetected and Quantified4.0-220 umol/mmol creatinineChildren (1-13 years old)Not SpecifiedNormal details
UrineDetected and Quantified3-78 umol/mmol creatinineChildren (1-13 years old)Not SpecifiedNormal details
UrineDetected and Quantified10-99 umol/mmol creatinineChildren (1-13 years old)BothNormal details
UrineDetected and Quantified<150 umol/mmol creatinineAdult (>18 years old)FemaleNormal details
UrineDetected and Quantified115.283 +/- 130.147 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
    • Mordechai, Hien, ...
details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
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
BloodDetected and Quantified17.3-27.0 uMAdult (>18 years old)FemaleCerebral creatine deficiency syndrome 2 details
BloodDetected and Quantified0.2-0.3 uMChildren (1-13 years old)FemaleCerebral creatine deficiency syndrome 3 details
BloodDetected and Quantified1.1 uMChildren (1-13 years old)MaleCerebral creatine deficiency syndrome 1 details
BloodDetected and Quantified3.280 +/- 1.230 uMAdult (>18 years old)Both
Uremia
details
Cerebrospinal Fluid (CSF)Detected and Quantified11.0-12.4 uMAdult (>18 years old)FemaleCerebral creatine deficiency syndrome 2 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 and Quantified614-1624 umol/mmol creatinineAdult (>18 years old)FemaleCerebral creatine deficiency syndrome 2 details
UrineDetected but not Quantified Adult (>18 years old)Both
Schizophrenia
details
UrineDetected and Quantified0.9-2.6 umol/mmol creatinineChildren (1-13 years old)FemaleCerebral creatine deficiency syndrome 3 details
UrineDetected and Quantified74 umol/mmol creatinineChildren (1-13 years old)MaleCerebral creatine deficiency syndrome 1 details
UrineDetected and Quantified55.592 +/- 42.373 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Eosinophilic esophagitis
    • Mordechai, Hien, ...
details
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 ]
Uremia
  1. Duranton F, Cohen G, De Smet R, Rodriguez M, Jankowski J, Vanholder R, Argiles A: Normal and pathologic concentrations of uremic toxins. J Am Soc Nephrol. 2012 Jul;23(7):1258-70. doi: 10.1681/ASN.2011121175. Epub 2012 May 24. [PubMed:22626821 ]
  2. Vanholder R, De Smet R, Glorieux G, Argiles A, Baurmeister U, Brunet P, Clark W, Cohen G, De Deyn PP, Deppisch R, Descamps-Latscha B, Henle T, Jorres A, Lemke HD, Massy ZA, Passlick-Deetjen J, Rodriguez M, Stegmayr B, Stenvinkel P, Tetta C, Wanner C, Zidek W: Review on uremic toxins: classification, concentration, and interindividual variability. Kidney Int. 2003 May;63(5):1934-43. doi: 10.1046/j.1523-1755.2003.00924.x. [PubMed:12675874 ]
Cerebral creatine deficiency syndrome 2
  1. Caldeira Araujo H, Smit W, Verhoeven NM, Salomons GS, Silva S, Vasconcelos R, Tomas H, Tavares de Almeida I, Jakobs C, Duran M: Guanidinoacetate methyltransferase deficiency identified in adults and a child with mental retardation. Am J Med Genet A. 2005 Mar 1;133A(2):122-7. [PubMed:15651030 ]
Cerebral creatine deficiency syndrome 3
  1. Nouioua S, Cheillan D, Zaouidi S, Salomons GS, Amedjout N, Kessaci F, Boulahdour N, Hamadouche T, Tazir M: Creatine deficiency syndrome. A treatable myopathy due to arginine-glycine amidinotransferase (AGAT) deficiency. Neuromuscul Disord. 2013 Aug;23(8):670-4. doi: 10.1016/j.nmd.2013.04.011. Epub 2013 Jun 14. [PubMed:23770102 ]
Cerebral creatine deficiency syndrome 1
  1. Salomons GS, van Dooren SJ, Verhoeven NM, Cecil KM, Ball WS, Degrauw TJ, Jakobs C: X-linked creatine-transporter gene (SLC6A8) defect: a new creatine-deficiency syndrome. Am J Hum Genet. 2001 Jun;68(6):1497-500. doi: 10.1086/320595. Epub 2001 Apr 20. [PubMed:11326334 ]
Crohn's disease
  1. Kolho KL, Pessia A, Jaakkola T, de Vos WM, Velagapudi V: Faecal and Serum Metabolomics in Paediatric Inflammatory Bowel Disease. J Crohns Colitis. 2017 Mar 1;11(3):321-334. doi: 10.1093/ecco-jcc/jjw158. [PubMed:27609529 ]
Ulcerative colitis
  1. Kolho KL, Pessia A, Jaakkola T, de Vos WM, Velagapudi V: Faecal and Serum Metabolomics in Paediatric Inflammatory Bowel Disease. J Crohns Colitis. 2017 Mar 1;11(3):321-334. doi: 10.1093/ecco-jcc/jjw158. [PubMed:27609529 ]
Eosinophilic esophagitis
  1. (). Mordechai, Hien, and David S. Wishart. .
Schizophrenia
  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
  • 612736 (Cerebral creatine deficiency syndrome 2)
  • 612718 (Cerebral creatine deficiency syndrome 3)
  • 300352 (Cerebral creatine deficiency syndrome 1)
  • 266600 (Crohn's disease)
  • 610247 (Eosinophilic esophagitis)
  • 181500 (Schizophrenia)
DrugBank IDDB02751
Phenol Explorer Compound IDNot Available
FoodDB IDFDB021898
KNApSAcK IDNot Available
Chemspider ID743
KEGG Compound IDC00581
BioCyc IDGUANIDOACETIC_ACID
BiGG ID1594816
Wikipedia LinkGlycocyamine
METLIN ID5163
PubChem Compound763
PDB IDNot Available
ChEBI ID16344
Food Biomarker OntologyNot Available
VMH IDNot Available
References
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. Schulze A: Creatine deficiency syndromes. Mol Cell Biochem. 2003 Feb;244(1-2):143-50. [PubMed:12701824 ]
  2. 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 ]
  3. 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 ]
  4. 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 ]
  5. 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 ]
  6. 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 ]
  7. 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 ]
  8. 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 ]
  9. 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 ]
  10. 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 ]
  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 ]
  15. Duranton F, Cohen G, De Smet R, Rodriguez M, Jankowski J, Vanholder R, Argiles A: Normal and pathologic concentrations of uremic toxins. J Am Soc Nephrol. 2012 Jul;23(7):1258-70. doi: 10.1681/ASN.2011121175. Epub 2012 May 24. [PubMed:22626821 ]

Enzymes

General function:
Involved in guanidinoacetate N-methyltransferase activity
Specific function:
Not Available
Gene Name:
GAMT
Uniprot ID:
Q14353
Molecular weight:
26317.925
Reactions
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:
GATM
Uniprot ID:
P50440
Molecular weight:
48455.01
Reactions
L-Arginine + Glycine → Ornithine + Guanidoacetic aciddetails