Showing Metabocard for guanidinoacetate (BASm0002928)

Common NameGuanidinoacetate
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
Molecular FormulaC3H7N3O2
Average Mass117.10660
Monoisotopic Mass117.05383
IUPAC Name2-carbamimidamidoacetic acid
Traditional NameGlycocyamine
CAS Registry Number352-97-6
SMILESNC(=[NH2+])NCC(=O)[O-]
InChI IdentifierInChI=1S/C3H7N3O2/c4-3(5)6-1-2(7)8/h1H2,(H,7,8)(H4,4,5,6)
InChI KeyBPMFZUMJYQTVII-UHFFFAOYSA-N
CHEBI IDCHEBI:57742
HMDB IDHMDB0000128
MiMeDB IDMMDBc0000496
Pathways
NameSMPDB/PathBank
Arginine and proline metabolism
Glycine, serine and threonine metabolism
Dihydropyrimidine Dehydrogenase Deficiency (DHPD)
Guanidinoacetate Methyltransferase Deficiency (GAMT Deficiency)
Prolidase Deficiency (PD)
Prolinemia Type II
Non Ketotic Hyperglycinemia
Dimethylglycine Dehydrogenase Deficiency
Sarcosinemia
Hyperprolinemia Type II
Hyperprolinemia Type I
Arginine: Glycine Amidinotransferase Deficiency (AGAT Deficiency)
Ornithine Aminotransferase Deficiency (OAT Deficiency)
Dimethylglycine Dehydrogenase Deficiency
Hyperglycinemia, non-ketotic
Creatine deficiency, guanidinoacetate methyltransferase deficiency
Hyperornithinemia with gyrate atrophy (HOGA)
Hyperornithinemia-hyperammonemia-homocitrullinuria [HHH-syndrome]
L-arginine:glycine amidinotransferase deficiency
3-Phosphoglycerate dehydrogenase deficiency
StateSolid
Water Solubility4.19e+00 g/l
logP-1.76
logS-1.45
pKa (Strongest Acidic)3.37
pKa (Strongest Basic)12.24
Hydrogen Acceptor Count5
Hydrogen Donor Count4
Polar Surface Area99.2 Ų
Rotatable Bond Count2
Physiological Charge0
Formal Charge0
Refractivity36.72 m³·mol⁻¹
Polarizability10.53

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