UniProt Protein Function: | Sulfurates 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. |
NCBI Summary: | This gene encodes an enzyme that sulfurates the molybdenum cofactor which is required for activation of the xanthine dehydrogenase (XDH) and aldehyde oxidase (AO) enzymes. XDH catalyzes the conversion of hypoxanthine to uric acid via xanthine, as well as the conversion of allopurinol to oxypurinol, and pyrazinamide to 5-hydroxy pyrazinamide. Mutations in this gene cause the metabolic disorder classical xanthinuria type II which is characterized by the loss of XDH/XO and AO enzyme activity, decreased levels of uric acid in the urine, increased levels of xanthine and hypoxanthine in the serum and urine, formation of xanthine stones in the urinary tract, and myositis due to tissue deposition of xanthine. [provided by RefSeq, Apr 2017] |
UniProt Code: | Q96EN8 |
NCBI GenInfo Identifier: | 157388923 |
NCBI Gene ID: | 55034 |
NCBI Accession: | NP_060417.2 |
UniProt Secondary Accession: | Q96EN8,Q53GP5, Q8N3A4, Q9NWM7, |
UniProt Related Accession: | Q96EN8 |
Molecular Weight: | Calculated: 98kDaObserved: 100kDa |
NCBI Full Name: | molybdenum cofactor sulfurase |
NCBI Synonym Full Names: | molybdenum cofactor sulfurase |
NCBI Official Symbol: | MOCOSÂ Â |
NCBI Official Synonym Symbols: | MCS; MOS; HMCSÂ Â |
NCBI Protein Information: | molybdenum cofactor sulfurase |
UniProt Protein Name: | Molybdenum cofactor sulfurase |
UniProt Synonym Protein Names: | Molybdenum cofactor sulfurtransferase |
UniProt Gene Name: | MOCOSÂ Â |