Family

Guanidinoacetate N-methyltransferase (IPR016550)

Short name: GuanidinoAc_N-MeTrfase

Family relationships

None.

Description

Guanidinoacetate methyltransferase (GAMT; EC:2.1.1.2) catalyses the last step of creatine biosynthesis. In humans, GAMT is believed to be the major enzyme involved in the metabolic conversion of S-adenosylmethionine (SAM) to S-adenosylhomocycteine (SAH). GAMT is found in abundance in the liver of all vertebrates. The crystal structure of a truncated version of GAMT shows that it exists as a dimer [PMID: 9325156], and the structure can be viewed as a ternary complex between protein arginine methyltransferase (one subunit) complexed with a protein substrate (the partner subunit) and the product SAH [PMID: 12079381]. Deficiency of GAMT in the liver causes a hereditary disease with extra pyramidal motor disorder and extremely low concentrations of creatine in the brain, serum and urine [PMID: 7808840].

GO terms

Biological Process

No terms assigned in this category.

Molecular Function

GO:0030731 guanidinoacetate N-methyltransferase activity

Cellular Component

No terms assigned in this category.

Contributing signatures

Signatures from InterPro member databases are used to construct an entry.
PIRSF