Glycine amidinotransferase, mitochondrial

UniProtKB accession:  P50440

Grouped By:  Matching UniProtKB accession

UniProtKB description:  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:3800397, PubMed:16820567, PubMed:36543883, PubMed:27233232). 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 (PubMed:3800397, PubMed:36543883, PubMed:9266688, PubMed:27233232) (Probable).