Biology:GATM (gene)

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A representation of the 3D structure of the protein myoglobin showing turquoise α-helices.
Generic protein structure example

Glycine amidinotransferase, mitochondrial is an enzyme that in humans is encoded by the GATM gene.[1][2]

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 arginine:glycine amidinotransferase deficiency, an inborn error of creatine synthesis characterized by mental retardation, language impairment, and behavioral disorders.[2]

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