3ORH259314431ENSG00000130005ENSMUSG00000020150Q14353O35969NM_138924NM_000156NM_010255NM_001347119NP_000147NP_620279NP_001334048NP_034385Guanidinoacetate N-methyltransferase (EC 2.1.1.2) is an enzyme that catalyzes the chemical reaction and is encoded by gene GAMT located on chromosome 19p13.3.

This enzyme belongs to the family of transferases, specifically those transferring one-carbon group methyltransferases.

The protein encoded by this gene is a methyltransferase that converts guanidoacetate to creatine, using S-adenosylmethionine as the methyl donor.

Defects in this gene have been implicated in neurologic syndromes and muscular hypotonia, probably due to creatine deficiency and accumulation of guanidinoacetate in the brain of affected individuals.

[5] As of late 2007, 7 structures have been solved for this class of enzymes, with PDB accession codes 1KHH, 1P1B, 1P1C, 1XCJ, 1XCL, 1ZX0, and 2BLN.