Glycine N-methyltransferase
Function
Catalyzes the methylation of glycine by using S-adenosylmethionine (AdoMet) to form N-methylglycine (sarcosine) with the concomitant production of S-adenosylhomocysteine (AdoHcy), a reaction regulated by the binding of 5-methyltetrahydrofolate. Plays an important role in the regulation of methyl group metabolism by regulating the ratio between S-adenosyl-L-methionine and S-adenosyl-L-homocysteine.
Involvement in disease
Glycine N-methyltransferase deficiency
GNMT deficiency
The only clinical abnormalities in patients with this deficiency are mild hepatomegaly and chronic elevation of serum transaminases.
None
The disease is caused by variants affecting the gene represented in this entry.
Sequence Similarities
Belongs to the class I-like SAM-binding methyltransferase superfamily. Glycine N-methyltransferase family.
Tissue Specificity
Expressed only in liver, pancreas, and prostate.
Cellular localization
- Cytoplasm
Alternative names
Glycine N-methyltransferase, GNMT