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Record Information
Version5.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2023-05-30 20:55:54 UTC
HMDB IDHMDB0000128
Secondary Accession Numbers
  • HMDB00128
Metabolite Identification
Common NameGuanidoacetic acid
DescriptionGuanidoacetic acid (GAA), also known as guanidinoacetate or glycocyamine, 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. Manufactured guanidinoacetic acid is primarily used a feed additive approved by EFSA in poultry farming (for fattening), and pigs for fattening. Guanidoacetic acid exists naturally in all vertebrates. It is formed primarily in the kidneys by transferring the guanidine group of L-arginine to the amino acid glycine via the enzyme known as L-Arg:Gly-amidinotransferase (AGAT). In a further step, guanidinoacetate is methylated to generate creatine using S-adenosyl methionine (as the methyl donor) via the enzyme known as guanidinoacetate N-methyltransferase (GAMT). The resulting creatine is released into the bloodstream. Elevated levels of guanidoacetic acid are a characteristic of an inborn metabolic disorder known as Guanidinoacetate Methyltransferase (GAMT) Deficiency. GAMT converts guanidinoacetate to creatine and deficiency of this enzyme results in creatine depletion and accumulation of guanidinoacetate The disorder is transmitted in an autosomal recessive fashion and is localized to mutations on chromosome 19p13.3. GAMT deficiency is characterized by developmental arrest, medication-resistant epilepsy (myoclonic, generalized tonic-clonic, partial complex, atonic), severe speech impairment, progressive dystonia, dyskinesias, hypotonia, ataxia, and autistic-like behavior.
Structure
Data?1676999672
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 effectNot Available
Disposition
Process
Role
Physical Properties
StateSolid
Experimental Molecular Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water Solubility3.6 mg/mL at 15 °CNot Available
LogPNot AvailableNot Available
Experimental Chromatographic Properties

Experimental Collision Cross Sections

Adduct TypeData SourceCCS Value (Å2)Reference
[M+H]+MetCCS_train_pos124.84330932474
[M+H]+Not Available125.5http://allccs.zhulab.cn/database/detail?ID=AllCCS00000487
Predicted Molecular 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
BioavailabilityYesChemAxon
Rule of FiveYesChemAxon
Ghose FilterNoChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleNoChemAxon
Predicted Chromatographic Properties

Predicted Collision Cross Sections

PredictorAdduct TypeCCS Value (Å2)Reference
DarkChem[M+H]+124.46431661259
DarkChem[M-H]-118.36231661259
AllCCS[M+H]+128.35432859911
AllCCS[M-H]-122.82132859911
DeepCCS[M+H]+127.3930932474
DeepCCS[M-H]-124.55530932474
DeepCCS[M-2H]-160.91930932474
DeepCCS[M+Na]+135.95330932474
AllCCS[M+H]+128.432859911
AllCCS[M+H-H2O]+124.232859911
AllCCS[M+NH4]+132.232859911
AllCCS[M+Na]+133.432859911
AllCCS[M-H]-122.832859911
AllCCS[M+Na-2H]-126.132859911
AllCCS[M+HCOO]-129.832859911

Predicted Kovats Retention Indices

Underivatized

MetaboliteSMILESKovats RI ValueColumn TypeReference
Guanidoacetic acidNC(=N)NCC(O)=O2484.0Standard polar33892256
Guanidoacetic acidNC(=N)NCC(O)=O1303.2Standard non polar33892256
Guanidoacetic acidNC(=N)NCC(O)=O1854.2Semi standard non polar33892256

Derivatized

Derivative Name / StructureSMILESKovats RI ValueColumn TypeReference
Guanidoacetic acid,1TMS,isomer #1C[Si](C)(C)OC(=O)CNC(=N)N1584.4Semi standard non polar33892256
Guanidoacetic acid,1TMS,isomer #2C[Si](C)(C)NC(=N)NCC(=O)O1724.8Semi standard non polar33892256
Guanidoacetic acid,1TMS,isomer #3C[Si](C)(C)N=C(N)NCC(=O)O1614.7Semi standard non polar33892256
Guanidoacetic acid,1TMS,isomer #4C[Si](C)(C)N(CC(=O)O)C(=N)N1649.4Semi standard non polar33892256
Guanidoacetic acid,2TMS,isomer #1C[Si](C)(C)NC(=N)NCC(=O)O[Si](C)(C)C1743.8Semi standard non polar33892256
Guanidoacetic acid,2TMS,isomer #1C[Si](C)(C)NC(=N)NCC(=O)O[Si](C)(C)C1486.2Standard non polar33892256
Guanidoacetic acid,2TMS,isomer #1C[Si](C)(C)NC(=N)NCC(=O)O[Si](C)(C)C2879.8Standard polar33892256
Guanidoacetic acid,2TMS,isomer #2C[Si](C)(C)OC(=O)CN(C(=N)N)[Si](C)(C)C1642.1Semi standard non polar33892256
Guanidoacetic acid,2TMS,isomer #2C[Si](C)(C)OC(=O)CN(C(=N)N)[Si](C)(C)C1469.1Standard non polar33892256
Guanidoacetic acid,2TMS,isomer #2C[Si](C)(C)OC(=O)CN(C(=N)N)[Si](C)(C)C2770.8Standard polar33892256
Guanidoacetic acid,2TMS,isomer #3C[Si](C)(C)N=C(N)NCC(=O)O[Si](C)(C)C1644.8Semi standard non polar33892256
Guanidoacetic acid,2TMS,isomer #3C[Si](C)(C)N=C(N)NCC(=O)O[Si](C)(C)C1411.1Standard non polar33892256
Guanidoacetic acid,2TMS,isomer #3C[Si](C)(C)N=C(N)NCC(=O)O[Si](C)(C)C2748.0Standard polar33892256
Guanidoacetic acid,2TMS,isomer #4C[Si](C)(C)N(C(=N)NCC(=O)O)[Si](C)(C)C1833.0Semi standard non polar33892256
Guanidoacetic acid,2TMS,isomer #4C[Si](C)(C)N(C(=N)NCC(=O)O)[Si](C)(C)C1541.6Standard non polar33892256
Guanidoacetic acid,2TMS,isomer #4C[Si](C)(C)N(C(=N)NCC(=O)O)[Si](C)(C)C2821.7Standard polar33892256
Guanidoacetic acid,2TMS,isomer #5C[Si](C)(C)N=C(NCC(=O)O)N[Si](C)(C)C1705.2Semi standard non polar33892256
Guanidoacetic acid,2TMS,isomer #5C[Si](C)(C)N=C(NCC(=O)O)N[Si](C)(C)C1446.6Standard non polar33892256
Guanidoacetic acid,2TMS,isomer #5C[Si](C)(C)N=C(NCC(=O)O)N[Si](C)(C)C2821.7Standard polar33892256
Guanidoacetic acid,2TMS,isomer #6C[Si](C)(C)NC(=N)N(CC(=O)O)[Si](C)(C)C1748.5Semi standard non polar33892256
Guanidoacetic acid,2TMS,isomer #6C[Si](C)(C)NC(=N)N(CC(=O)O)[Si](C)(C)C1520.6Standard non polar33892256
Guanidoacetic acid,2TMS,isomer #6C[Si](C)(C)NC(=N)N(CC(=O)O)[Si](C)(C)C2683.8Standard polar33892256
Guanidoacetic acid,2TMS,isomer #7C[Si](C)(C)N=C(N)N(CC(=O)O)[Si](C)(C)C1674.1Semi standard non polar33892256
Guanidoacetic acid,2TMS,isomer #7C[Si](C)(C)N=C(N)N(CC(=O)O)[Si](C)(C)C1470.1Standard non polar33892256
Guanidoacetic acid,2TMS,isomer #7C[Si](C)(C)N=C(N)N(CC(=O)O)[Si](C)(C)C2687.2Standard polar33892256
Guanidoacetic acid,3TMS,isomer #1C[Si](C)(C)OC(=O)CNC(=N)N([Si](C)(C)C)[Si](C)(C)C1812.1Semi standard non polar33892256
Guanidoacetic acid,3TMS,isomer #1C[Si](C)(C)OC(=O)CNC(=N)N([Si](C)(C)C)[Si](C)(C)C1610.7Standard non polar33892256
Guanidoacetic acid,3TMS,isomer #1C[Si](C)(C)OC(=O)CNC(=N)N([Si](C)(C)C)[Si](C)(C)C2398.4Standard polar33892256
Guanidoacetic acid,3TMS,isomer #2C[Si](C)(C)N=C(NCC(=O)O[Si](C)(C)C)N[Si](C)(C)C1728.2Semi standard non polar33892256
Guanidoacetic acid,3TMS,isomer #2C[Si](C)(C)N=C(NCC(=O)O[Si](C)(C)C)N[Si](C)(C)C1404.3Standard non polar33892256
Guanidoacetic acid,3TMS,isomer #2C[Si](C)(C)N=C(NCC(=O)O[Si](C)(C)C)N[Si](C)(C)C2554.3Standard polar33892256
Guanidoacetic acid,3TMS,isomer #3C[Si](C)(C)NC(=N)N(CC(=O)O[Si](C)(C)C)[Si](C)(C)C1712.3Semi standard non polar33892256
Guanidoacetic acid,3TMS,isomer #3C[Si](C)(C)NC(=N)N(CC(=O)O[Si](C)(C)C)[Si](C)(C)C1591.7Standard non polar33892256
Guanidoacetic acid,3TMS,isomer #3C[Si](C)(C)NC(=N)N(CC(=O)O[Si](C)(C)C)[Si](C)(C)C2431.2Standard polar33892256
Guanidoacetic acid,3TMS,isomer #4C[Si](C)(C)N=C(N)N(CC(=O)O[Si](C)(C)C)[Si](C)(C)C1641.8Semi standard non polar33892256
Guanidoacetic acid,3TMS,isomer #4C[Si](C)(C)N=C(N)N(CC(=O)O[Si](C)(C)C)[Si](C)(C)C1458.6Standard non polar33892256
Guanidoacetic acid,3TMS,isomer #4C[Si](C)(C)N=C(N)N(CC(=O)O[Si](C)(C)C)[Si](C)(C)C2599.0Standard polar33892256
Guanidoacetic acid,3TMS,isomer #5C[Si](C)(C)N=C(NCC(=O)O)N([Si](C)(C)C)[Si](C)(C)C1776.6Semi standard non polar33892256
Guanidoacetic acid,3TMS,isomer #5C[Si](C)(C)N=C(NCC(=O)O)N([Si](C)(C)C)[Si](C)(C)C1575.1Standard non polar33892256
Guanidoacetic acid,3TMS,isomer #5C[Si](C)(C)N=C(NCC(=O)O)N([Si](C)(C)C)[Si](C)(C)C2452.9Standard polar33892256
Guanidoacetic acid,3TMS,isomer #6C[Si](C)(C)N(CC(=O)O)C(=N)N([Si](C)(C)C)[Si](C)(C)C1780.5Semi standard non polar33892256
Guanidoacetic acid,3TMS,isomer #6C[Si](C)(C)N(CC(=O)O)C(=N)N([Si](C)(C)C)[Si](C)(C)C1668.6Standard non polar33892256
Guanidoacetic acid,3TMS,isomer #6C[Si](C)(C)N(CC(=O)O)C(=N)N([Si](C)(C)C)[Si](C)(C)C2365.2Standard polar33892256
Guanidoacetic acid,3TMS,isomer #7C[Si](C)(C)N=C(N[Si](C)(C)C)N(CC(=O)O)[Si](C)(C)C1695.4Semi standard non polar33892256
Guanidoacetic acid,3TMS,isomer #7C[Si](C)(C)N=C(N[Si](C)(C)C)N(CC(=O)O)[Si](C)(C)C1533.2Standard non polar33892256
Guanidoacetic acid,3TMS,isomer #7C[Si](C)(C)N=C(N[Si](C)(C)C)N(CC(=O)O)[Si](C)(C)C2373.1Standard polar33892256
Guanidoacetic acid,4TMS,isomer #1C[Si](C)(C)OC(=O)CN(C(=N)N([Si](C)(C)C)[Si](C)(C)C)[Si](C)(C)C1757.8Semi standard non polar33892256
Guanidoacetic acid,4TMS,isomer #1C[Si](C)(C)OC(=O)CN(C(=N)N([Si](C)(C)C)[Si](C)(C)C)[Si](C)(C)C1737.2Standard non polar33892256
Guanidoacetic acid,4TMS,isomer #1C[Si](C)(C)OC(=O)CN(C(=N)N([Si](C)(C)C)[Si](C)(C)C)[Si](C)(C)C2168.4Standard polar33892256
Guanidoacetic acid,4TMS,isomer #2C[Si](C)(C)N=C(NCC(=O)O[Si](C)(C)C)N([Si](C)(C)C)[Si](C)(C)C1765.0Semi standard non polar33892256
Guanidoacetic acid,4TMS,isomer #2C[Si](C)(C)N=C(NCC(=O)O[Si](C)(C)C)N([Si](C)(C)C)[Si](C)(C)C1561.2Standard non polar33892256
Guanidoacetic acid,4TMS,isomer #2C[Si](C)(C)N=C(NCC(=O)O[Si](C)(C)C)N([Si](C)(C)C)[Si](C)(C)C2212.0Standard polar33892256
Guanidoacetic acid,4TMS,isomer #3C[Si](C)(C)N=C(N[Si](C)(C)C)N(CC(=O)O[Si](C)(C)C)[Si](C)(C)C1715.8Semi standard non polar33892256
Guanidoacetic acid,4TMS,isomer #3C[Si](C)(C)N=C(N[Si](C)(C)C)N(CC(=O)O[Si](C)(C)C)[Si](C)(C)C1525.0Standard non polar33892256
Guanidoacetic acid,4TMS,isomer #3C[Si](C)(C)N=C(N[Si](C)(C)C)N(CC(=O)O[Si](C)(C)C)[Si](C)(C)C2180.4Standard polar33892256
Guanidoacetic acid,4TMS,isomer #4C[Si](C)(C)N=C(N(CC(=O)O)[Si](C)(C)C)N([Si](C)(C)C)[Si](C)(C)C1792.2Semi standard non polar33892256
Guanidoacetic acid,4TMS,isomer #4C[Si](C)(C)N=C(N(CC(=O)O)[Si](C)(C)C)N([Si](C)(C)C)[Si](C)(C)C1712.6Standard non polar33892256
Guanidoacetic acid,4TMS,isomer #4C[Si](C)(C)N=C(N(CC(=O)O)[Si](C)(C)C)N([Si](C)(C)C)[Si](C)(C)C2011.1Standard polar33892256
Guanidoacetic acid,5TMS,isomer #1C[Si](C)(C)N=C(N(CC(=O)O[Si](C)(C)C)[Si](C)(C)C)N([Si](C)(C)C)[Si](C)(C)C1809.1Semi standard non polar33892256
Guanidoacetic acid,5TMS,isomer #1C[Si](C)(C)N=C(N(CC(=O)O[Si](C)(C)C)[Si](C)(C)C)N([Si](C)(C)C)[Si](C)(C)C1724.7Standard non polar33892256
Guanidoacetic acid,5TMS,isomer #1C[Si](C)(C)N=C(N(CC(=O)O[Si](C)(C)C)[Si](C)(C)C)N([Si](C)(C)C)[Si](C)(C)C1848.3Standard polar33892256
Guanidoacetic acid,1TBDMS,isomer #1CC(C)(C)[Si](C)(C)OC(=O)CNC(=N)N1835.5Semi standard non polar33892256
Guanidoacetic acid,1TBDMS,isomer #2CC(C)(C)[Si](C)(C)NC(=N)NCC(=O)O1980.4Semi standard non polar33892256
Guanidoacetic acid,1TBDMS,isomer #3CC(C)(C)[Si](C)(C)N=C(N)NCC(=O)O1859.7Semi standard non polar33892256
Guanidoacetic acid,1TBDMS,isomer #4CC(C)(C)[Si](C)(C)N(CC(=O)O)C(=N)N1875.6Semi standard non polar33892256
Guanidoacetic acid,2TBDMS,isomer #1CC(C)(C)[Si](C)(C)NC(=N)NCC(=O)O[Si](C)(C)C(C)(C)C2218.2Semi standard non polar33892256
Guanidoacetic acid,2TBDMS,isomer #1CC(C)(C)[Si](C)(C)NC(=N)NCC(=O)O[Si](C)(C)C(C)(C)C1846.5Standard non polar33892256
Guanidoacetic acid,2TBDMS,isomer #1CC(C)(C)[Si](C)(C)NC(=N)NCC(=O)O[Si](C)(C)C(C)(C)C2749.2Standard polar33892256
Guanidoacetic acid,2TBDMS,isomer #2CC(C)(C)[Si](C)(C)OC(=O)CN(C(=N)N)[Si](C)(C)C(C)(C)C2079.4Semi standard non polar33892256
Guanidoacetic acid,2TBDMS,isomer #2CC(C)(C)[Si](C)(C)OC(=O)CN(C(=N)N)[Si](C)(C)C(C)(C)C1865.8Standard non polar33892256
Guanidoacetic acid,2TBDMS,isomer #2CC(C)(C)[Si](C)(C)OC(=O)CN(C(=N)N)[Si](C)(C)C(C)(C)C2822.9Standard polar33892256
Guanidoacetic acid,2TBDMS,isomer #3CC(C)(C)[Si](C)(C)N=C(N)NCC(=O)O[Si](C)(C)C(C)(C)C2073.2Semi standard non polar33892256
Guanidoacetic acid,2TBDMS,isomer #3CC(C)(C)[Si](C)(C)N=C(N)NCC(=O)O[Si](C)(C)C(C)(C)C1754.0Standard non polar33892256
Guanidoacetic acid,2TBDMS,isomer #3CC(C)(C)[Si](C)(C)N=C(N)NCC(=O)O[Si](C)(C)C(C)(C)C2824.4Standard polar33892256
Guanidoacetic acid,2TBDMS,isomer #4CC(C)(C)[Si](C)(C)N(C(=N)NCC(=O)O)[Si](C)(C)C(C)(C)C2234.0Semi standard non polar33892256
Guanidoacetic acid,2TBDMS,isomer #4CC(C)(C)[Si](C)(C)N(C(=N)NCC(=O)O)[Si](C)(C)C(C)(C)C1930.3Standard non polar33892256
Guanidoacetic acid,2TBDMS,isomer #4CC(C)(C)[Si](C)(C)N(C(=N)NCC(=O)O)[Si](C)(C)C(C)(C)C2636.5Standard polar33892256
Guanidoacetic acid,2TBDMS,isomer #5CC(C)(C)[Si](C)(C)N=C(NCC(=O)O)N[Si](C)(C)C(C)(C)C2209.7Semi standard non polar33892256
Guanidoacetic acid,2TBDMS,isomer #5CC(C)(C)[Si](C)(C)N=C(NCC(=O)O)N[Si](C)(C)C(C)(C)C1749.5Standard non polar33892256
Guanidoacetic acid,2TBDMS,isomer #5CC(C)(C)[Si](C)(C)N=C(NCC(=O)O)N[Si](C)(C)C(C)(C)C2631.2Standard polar33892256
Guanidoacetic acid,2TBDMS,isomer #6CC(C)(C)[Si](C)(C)NC(=N)N(CC(=O)O)[Si](C)(C)C(C)(C)C2204.0Semi standard non polar33892256
Guanidoacetic acid,2TBDMS,isomer #6CC(C)(C)[Si](C)(C)NC(=N)N(CC(=O)O)[Si](C)(C)C(C)(C)C1922.2Standard non polar33892256
Guanidoacetic acid,2TBDMS,isomer #6CC(C)(C)[Si](C)(C)NC(=N)N(CC(=O)O)[Si](C)(C)C(C)(C)C2599.3Standard polar33892256
Guanidoacetic acid,2TBDMS,isomer #7CC(C)(C)[Si](C)(C)N=C(N)N(CC(=O)O)[Si](C)(C)C(C)(C)C2072.4Semi standard non polar33892256
Guanidoacetic acid,2TBDMS,isomer #7CC(C)(C)[Si](C)(C)N=C(N)N(CC(=O)O)[Si](C)(C)C(C)(C)C1795.5Standard non polar33892256
Guanidoacetic acid,2TBDMS,isomer #7CC(C)(C)[Si](C)(C)N=C(N)N(CC(=O)O)[Si](C)(C)C(C)(C)C2741.3Standard polar33892256
Guanidoacetic acid,3TBDMS,isomer #1CC(C)(C)[Si](C)(C)OC(=O)CNC(=N)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C2399.0Semi standard non polar33892256
Guanidoacetic acid,3TBDMS,isomer #1CC(C)(C)[Si](C)(C)OC(=O)CNC(=N)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C2197.7Standard non polar33892256
Guanidoacetic acid,3TBDMS,isomer #1CC(C)(C)[Si](C)(C)OC(=O)CNC(=N)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C2533.7Standard polar33892256
Guanidoacetic acid,3TBDMS,isomer #2CC(C)(C)[Si](C)(C)N=C(NCC(=O)O[Si](C)(C)C(C)(C)C)N[Si](C)(C)C(C)(C)C2335.8Semi standard non polar33892256
Guanidoacetic acid,3TBDMS,isomer #2CC(C)(C)[Si](C)(C)N=C(NCC(=O)O[Si](C)(C)C(C)(C)C)N[Si](C)(C)C(C)(C)C1919.2Standard non polar33892256
Guanidoacetic acid,3TBDMS,isomer #2CC(C)(C)[Si](C)(C)N=C(NCC(=O)O[Si](C)(C)C(C)(C)C)N[Si](C)(C)C(C)(C)C2562.8Standard polar33892256
Guanidoacetic acid,3TBDMS,isomer #3CC(C)(C)[Si](C)(C)NC(=N)N(CC(=O)O[Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C2375.4Semi standard non polar33892256
Guanidoacetic acid,3TBDMS,isomer #3CC(C)(C)[Si](C)(C)NC(=N)N(CC(=O)O[Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C2166.6Standard non polar33892256
Guanidoacetic acid,3TBDMS,isomer #3CC(C)(C)[Si](C)(C)NC(=N)N(CC(=O)O[Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C2556.0Standard polar33892256
Guanidoacetic acid,3TBDMS,isomer #4CC(C)(C)[Si](C)(C)N=C(N)N(CC(=O)O[Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C2259.8Semi standard non polar33892256
Guanidoacetic acid,3TBDMS,isomer #4CC(C)(C)[Si](C)(C)N=C(N)N(CC(=O)O[Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C2002.5Standard non polar33892256
Guanidoacetic acid,3TBDMS,isomer #4CC(C)(C)[Si](C)(C)N=C(N)N(CC(=O)O[Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C2786.2Standard polar33892256
Guanidoacetic acid,3TBDMS,isomer #5CC(C)(C)[Si](C)(C)N=C(NCC(=O)O)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C2370.1Semi standard non polar33892256
Guanidoacetic acid,3TBDMS,isomer #5CC(C)(C)[Si](C)(C)N=C(NCC(=O)O)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C2083.1Standard non polar33892256
Guanidoacetic acid,3TBDMS,isomer #5CC(C)(C)[Si](C)(C)N=C(NCC(=O)O)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C2482.3Standard polar33892256
Guanidoacetic acid,3TBDMS,isomer #6CC(C)(C)[Si](C)(C)N(CC(=O)O)C(=N)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C2384.2Semi standard non polar33892256
Guanidoacetic acid,3TBDMS,isomer #6CC(C)(C)[Si](C)(C)N(CC(=O)O)C(=N)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C2272.8Standard non polar33892256
Guanidoacetic acid,3TBDMS,isomer #6CC(C)(C)[Si](C)(C)N(CC(=O)O)C(=N)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C2497.0Standard polar33892256
Guanidoacetic acid,3TBDMS,isomer #7CC(C)(C)[Si](C)(C)N=C(N[Si](C)(C)C(C)(C)C)N(CC(=O)O)[Si](C)(C)C(C)(C)C2322.9Semi standard non polar33892256
Guanidoacetic acid,3TBDMS,isomer #7CC(C)(C)[Si](C)(C)N=C(N[Si](C)(C)C(C)(C)C)N(CC(=O)O)[Si](C)(C)C(C)(C)C2002.9Standard non polar33892256
Guanidoacetic acid,3TBDMS,isomer #7CC(C)(C)[Si](C)(C)N=C(N[Si](C)(C)C(C)(C)C)N(CC(=O)O)[Si](C)(C)C(C)(C)C2457.3Standard polar33892256
Guanidoacetic acid,4TBDMS,isomer #1CC(C)(C)[Si](C)(C)OC(=O)CN(C(=N)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C2590.4Semi standard non polar33892256
Guanidoacetic acid,4TBDMS,isomer #1CC(C)(C)[Si](C)(C)OC(=O)CN(C(=N)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C2485.2Standard non polar33892256
Guanidoacetic acid,4TBDMS,isomer #1CC(C)(C)[Si](C)(C)OC(=O)CN(C(=N)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C2487.6Standard polar33892256
Guanidoacetic acid,4TBDMS,isomer #2CC(C)(C)[Si](C)(C)N=C(NCC(=O)O[Si](C)(C)C(C)(C)C)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C2564.0Semi standard non polar33892256
Guanidoacetic acid,4TBDMS,isomer #2CC(C)(C)[Si](C)(C)N=C(NCC(=O)O[Si](C)(C)C(C)(C)C)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C2264.6Standard non polar33892256
Guanidoacetic acid,4TBDMS,isomer #2CC(C)(C)[Si](C)(C)N=C(NCC(=O)O[Si](C)(C)C(C)(C)C)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C2466.5Standard polar33892256
Guanidoacetic acid,4TBDMS,isomer #3CC(C)(C)[Si](C)(C)N=C(N[Si](C)(C)C(C)(C)C)N(CC(=O)O[Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C2499.5Semi standard non polar33892256
Guanidoacetic acid,4TBDMS,isomer #3CC(C)(C)[Si](C)(C)N=C(N[Si](C)(C)C(C)(C)C)N(CC(=O)O[Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C2204.3Standard non polar33892256
Guanidoacetic acid,4TBDMS,isomer #3CC(C)(C)[Si](C)(C)N=C(N[Si](C)(C)C(C)(C)C)N(CC(=O)O[Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C2461.6Standard polar33892256
Guanidoacetic acid,4TBDMS,isomer #4CC(C)(C)[Si](C)(C)N=C(N(CC(=O)O)[Si](C)(C)C(C)(C)C)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C2574.1Semi standard non polar33892256
Guanidoacetic acid,4TBDMS,isomer #4CC(C)(C)[Si](C)(C)N=C(N(CC(=O)O)[Si](C)(C)C(C)(C)C)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C2383.4Standard non polar33892256
Guanidoacetic acid,4TBDMS,isomer #4CC(C)(C)[Si](C)(C)N=C(N(CC(=O)O)[Si](C)(C)C(C)(C)C)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C2354.5Standard polar33892256
Guanidoacetic acid,5TBDMS,isomer #1CC(C)(C)[Si](C)(C)N=C(N(CC(=O)O[Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C2797.0Semi standard non polar33892256
Guanidoacetic acid,5TBDMS,isomer #1CC(C)(C)[Si](C)(C)N=C(N(CC(=O)O[Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C2593.3Standard non polar33892256
Guanidoacetic acid,5TBDMS,isomer #1CC(C)(C)[Si](C)(C)N=C(N(CC(=O)O[Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C)N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C2350.8Standard polar33892256
Spectra
Biological Properties
Cellular Locations
  • Cytoplasm (predicted from logP)
Biospecimen Locations
  • Blood
  • Cellular Cytoplasm
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Urine
Tissue Locations
  • Brain
  • Kidney
  • Liver
  • Placenta
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified2.8 +/- 0.9 uMAdult (>18 years old)BothNormal details
BloodDetected but not QuantifiedNot QuantifiedAdult (>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 QuantifiedNot QuantifiedChildren (6 - 18 years old)Not SpecifiedNormal details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
UrineDetected and Quantified127.26 umol/mmol creatinineNewborn (0-30 days old)Both
Normal
details
UrineDetected and Quantified35-259 umol/mmol creatinineNewborn (0-30 days old)Both
Normal
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 Quantified11.30 (7.28-20.30) umol/mmol creatinineNewborn (0-30 days old)Both
Normal
    • Analysis of 40 NI...
details
UrineDetected and Quantified13.3 (5.2-21.5) umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected but not QuantifiedNot QuantifiedAdult (>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 Quantified93-190 umol/mmol creatinineNewborn (0-30 days old)Both
Normal
details
UrineDetected and Quantified115.283 +/- 130.147 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
    • Analysis of 30 no...
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 QuantifiedNot QuantifiedChildren (6 - 18 years old)Not SpecifiedCrohns disease details
FecesDetected but not QuantifiedNot QuantifiedChildren (6 - 18 years old)Not SpecifiedUlcerative colitis details
FecesDetected but not QuantifiedNot QuantifiedChildren (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 QuantifiedNot QuantifiedAdult (>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
    • Analysis of 30 no...
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. Slae, M., Huynh, H., Wishart, D.S. (2014). Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work). NA.
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
FooDB IDFDB005417
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 IDGUDAC
MarkerDB IDMDB00013419
Good Scents 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. 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 ]
  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 ]
  16. Elshenawy S, Pinney SE, Stuart T, Doulias PT, Zura G, Parry S, Elovitz MA, Bennett MJ, Bansal A, Strauss JF 3rd, Ischiropoulos H, Simmons RA: The Metabolomic Signature of the Placenta in Spontaneous Preterm Birth. Int J Mol Sci. 2020 Feb 4;21(3). pii: ijms21031043. doi: 10.3390/ijms21031043. [PubMed:32033212 ]

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