The GATM gene provides instructions for making the enzyme arginine:glycine amidinotransferase. This enzyme participates in the two-step production (synthesis) of the compound creatine from the protein building blocks (amino acids) glycine, arginine, and methionine. Specifically, arginine:glycine amidinotransferase controls the first step of the process. In this step, a compound called guanidinoacetic acid is produced by transferring a cluster of nitrogen and hydrogen atoms called a guanidino group from arginine to glycine. Guanidinoacetic acid is converted to creatine in the second step of the process. Creatine is needed for the body to store and use energy properly.
Other Names
AGAT; AT; GATM_HUMAN; glycine amidinotransferase (L-arginine:glycine amidinotransferase); glycine amidinotransferase, mitochondrial; glycine amidinotransferase, mitochondrial precursor; L-arginine:glycine amidinotransferase; transamidinase
Related Genetics Topics
Identifiers
- NCBI Gene: 2628
- OMIM: 602360
Source: MedlinePlus Genetics, U.S. National Library of Medicine.
