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MGP005361

UniProt Annotations

Entry Information
Gene Namemolybdenum cofactor sulfurase
Protein EntryMOCOS_HUMAN
UniProt IDQ96EN8
SpeciesHuman
Comments
Comment typeDescription
Biophysicochemical PropertiesKinetic parameters: KM=15 uM for benzamidoxime {ECO:0000269|PubMed:16973608}; Vmax=0.24 nmol/min/mg enzyme {ECO:0000269|PubMed:16973608};
Catalytic ActivityMolybdenum cofactor + L-cysteine + 2 H(+) = thio-molybdenum cofactor + L-alanine + H(2)O. {ECO:0000255|HAMAP- Rule:MF_03050}.
CofactorName=pyridoxal 5'-phosphate; Xref=ChEBI:CHEBI:597326; Evidence={ECO:0000255|HAMAP-Rule:MF_03050};
DiseaseXanthinuria 2 (XU2) [MIM:603592]: A disorder characterized by excretion of very large amounts of xanthine in the urine and a tendency to form xanthine stones. Uric acid is strikingly diminished in serum and urine. In addition, patients suffering of xanthinuria 2 cannot metabolize allopurinol into oxypurinol due to dual deficiency of xanthine dehydrogenase and aldehyde oxidase. {ECO:0000269|PubMed:11302742, ECO:0000269|PubMed:14624414, ECO:0000269|PubMed:17368066, ECO:0000269|Ref.10}. Note=The disease is caused by mutations affecting the gene represented in this entry.
FunctionSulfurates the molybdenum cofactor. Sulfation of molybdenum is essential for xanthine dehydrogenase (XDH) and aldehyde oxidase (ADO) enzymes in which molybdenum cofactor is liganded by 1 oxygen and 1 sulfur atom in active form. In vitro, the C-terminal domain is able to reduce N-hydroxylated prodrugs, such as benzamidoxime. {ECO:0000255|HAMAP-Rule:MF_03050, ECO:0000269|PubMed:16973608}.
SimilarityBelongs to the class-V pyridoxal-phosphate-dependent aminotransferase family. MOCOS subfamily. {ECO:0000255|HAMAP- Rule:MF_03050}.
SimilarityContains 1 MOSC domain. {ECO:0000255|HAMAP- Rule:MF_03050}.
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