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molybdenum cofactor sulfurase (MoCo sulfurase, MOS, HMCS, MOCOS)

Function: 1) sulfurates molybdenum cofactor 2) sulfation of molybdenum is essential for xanthine dehydrogenase & aldehyde oxidase in which molybdenum cofactor is liganded by 1 oxygen & 1 sulfur atom in active form Cofactor: pyridoxal phosphate Pathology: - defects in MOCOS are the cause of xanthinuria type 2

General

lyase phosphoprotein

Properties

SIZE: MW = 98 kD entity length = 888 aa MOTIF: cofactor-binding site [264-264] FOR-BINDING-OF: pyridoxal phosphate cysteine residue {C424} Ser phosphorylation site {S528} Ser phosphorylation site {S530}

Database Correlations

OMIM 603592 UniProt Q96EN8 PFAM correlations

References

UniProt :accession Q96EN8