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MGP001871

UniProt Annotations

Entry Information
Gene Namemethionine adenosyltransferase I, alpha
Protein EntryMETK1_HUMAN
UniProt IDQ00266
SpeciesHuman
Comments
Comment typeDescription
Catalytic ActivityATP + L-methionine + H(2)O = phosphate + diphosphate + S-adenosyl-L-methionine.
CofactorName=K(+); Xref=ChEBI:CHEBI:29103; Evidence={ECO:0000250}; Note=Binds 1 potassium ion per subunit. {ECO:0000250};
CofactorName=Mg(2+); Xref=ChEBI:CHEBI:18420; Evidence={ECO:0000250}; Name=Co(2+); Xref=ChEBI:CHEBI:48828; Evidence={ECO:0000250}; Note=Binds 2 divalent ions per subunit. Magnesium or cobalt. {ECO:0000250};
DiseaseMethionine adenosyltransferase deficiency (MATD) [MIM:250850]: 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. {ECO:0000269|PubMed:10677294, ECO:0000269|PubMed:7560086, ECO:0000269|PubMed:8770875, ECO:0000269|PubMed:9042912}. Note=The disease is caused by mutations affecting the gene represented in this entry.
FunctionCatalyzes the formation of S-adenosylmethionine from methionine and ATP.
PathwayAmino-acid biosynthesis; S-adenosyl-L-methionine biosynthesis; S-adenosyl-L-methionine from L-methionine: step 1/1.
PtmS-nitrosylation of Cys-120 inactivates the enzyme. {ECO:0000250}.
SimilarityBelongs to the AdoMet synthase family. {ECO:0000305}.
SubunitHomotetramer (MAT-I) or homodimer (MAT-III). {ECO:0000269|Ref.7}.
Tissue SpecificityExpressed in liver.
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