GATM

Glycine amidinotransferase, mitochondrial; Isoform 2 of Glycine amidinotransferase, mitochondrial;Glycine amidinotransferase, mitochondrial

Transamidinase that catalyzes the transfer of the amidino group of L-arginine onto the amino moiety of acceptor metabolites such as glycine, beta-alanine, gamma-aminobutyric acid (GABA) and taurine yielding the corresponding guanidine derivatives (PubMed:16820567, PubMed:27233232, PubMed:36543883, PubMed:3800397). Catalyzes the rate-limiting step of creatine biosynthesis, namely the transfer of the amidino group from L-arginine to glycine to generate guanidinoacetate, which is then methylated by GAMT to form creatine. Provides creatine as a source for ATP generation in tissues with high energy demands, in particular skeletal muscle, heart and brain (Probable) (PubMed:27233232, PubMed:36543883, PubMed:3800397, PubMed:9266688).

Matrix Type

  • Plasma
  • Tissue/Cells

Gene Symbol

  • GATM

UniProt ID

  • P50440

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