MAT1A

Function

Catalyzes the formation of S-adenosylmethionine from methionine and ATP. The reaction comprises two steps that are both catalyzed by the same enzyme: formation of S-adenosylmethionine (AdoMet) and triphosphate, and subsequent hydrolysis of the triphosphate.

Involvement in disease

Methionine adenosyltransferase deficiency

MATD

An inborn error of metabolism resulting in isolated hypermethioninemia. Most patients have no clinical abnormalities, although some neurologic symptoms may be present in rare cases with severe loss of methionine adenosyltransferase activity.

None

The disease is caused by variants affecting the gene represented in this entry.

Pathway

Amino-acid biosynthesis; S-adenosyl-L-methionine biosynthesis; S-adenosyl-L-methionine from L-methionine: step 1/1.

Post-translational modifications

S-nitrosylation of Cys-120 inactivates the enzyme.

An intrachain disulfide bond can be formed. The protein structure shows that the relevant Cys residues are in a position that would permit formation of a disulfide bond.

Sequence Similarities

Belongs to the AdoMet synthase family.

Tissue Specificity

Expressed in liver.

Alternative names

AMS1, MATA1, MAT1A, S-adenosylmethionine synthase isoform type-1, AdoMet synthase 1, Methionine adenosyltransferase 1, Methionine adenosyltransferase I/III, MAT 1, MAT-I/III

Database links

swissprot:Q00266 omim:601468 omim:610550 swissprot:P31153 entrezGene:4143 entrezGene:4144