[31] The first two errors appear in creatine biosynthesis as autosomal recessive deficiencies of arginine-glycine
amidinotransferase (AGAT) and guanidinoacetate methyltransferase (GAMT).
A creatina e um nutriente natural, de procedencia animal encontrada em carnes vermelhas e peixes (Gualano e colaboradores, 2008), derivado de tres aminoacidos: glicina, arginina e metionina (Torres-Leal e Marreiro, 2008; Cooper e colaboradores, 2012) e tres enzimas: L-arginina-glicina
amidinotransferase (AGAT), metionina adenosiltransferase (MAT) e guanidinoacetato metiltransferase (GAMT) que sao indispensaveis para a sintese de creatina (Cooper e colaboradores, 2012).
Guanidinoacetate and creatine plus creatinine assessment in physiologic fluids: an effective diagnostic tool for the biochemical diagnosis of arginine:glycine
amidinotransferase and guanidinoacetate methyltransferase deficiencies.
Decreased GAA, decreased GAA/crn ratio, and increased CRE/GAA ratio define the biochemical phenotype of arginine:glycine
amidinotransferase (AGAT) deficiency.
It is well known that the transfer of the amidino group of arginine to glycine to yield L-ornithine and guanidinoacetate (GM), which is catalyzed by L-arginine:glycine
amidinotransferase, represents the first of two steps in the biosynthesis of Cr.
The amino acid L-arginine is substrate to five groups of enzymes in mammalians (Figure 5), which include arginyltRNA synthetase, arginine:glycine
amidinotransferase, arginine decarboxylase, arginase, and nitric oxide synthase isoforms [22].
rat kidney L-arginine:glycine
amidinotransferase synthesis by creatine
(14) Additional branches of arg metabolism are mediated by: (a) nitric oxide synthases (NOSs) leading to production of nitric oxide (NO) and downstream nitrogenous compounds, (b) arg decarboxylase leading to agmatine and its related byproducts, and (c) arg:glycine
amidinotransferase leading to guanidinoacetic acid, the immediate precursor of creatine.
For instance, among identified targets were titan and mitochondrial glycine
amidinotransferase, two proteins known to occur in the low nanogram per milliliter range (4-6.8 ng/mL) in blood (Farrah a al.
These are deficiencies of arginine:glycine
amidinotransferase (EC 2.1.4.1), S-adenosyl-L-methionine:guanidinoacetate N-methyltransferase (GAMT; EC 2.1.1.2), and creatine transporter.
In the current study, the gene encoding L-arginine-glycine
amidinotransferase, a member of the creatinine biosynthesis pathway, was affected as early as the first day after treatment with cisplatin.