GATM (gene)
Glycine amidinotransferase, mitochondrial is an enzyme that in humans is encoded by the GATM gene.
This gene encodes a mitochondrial enzyme that belongs to the Amidinotransferase family. This enzyme is involved in creatine biosynthesis, whereby it catalyzes the transfer of a guanido group from L-arginine to glycine, resulting in guanidinoacetic acid, the immediate precursor of creatine. Mutations in this gene cause, an inborn error of creatine synthesis characterized by mental retardation, language impairment, and behavioral disorders.
