Recombinant Human Molybdenum cofactor sulfurase (MOCOS), Truncated

Uniprot Id

Q96EN8

Target Species

Human

Target Name

MOCOS

Target Full Name

Molybdenum cofactor sulfurase

Target 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.

Target Involvement

Xanthinuria 2 (XAN2)

Target Protein Families

Class-V pyridoxal-phosphate-dependent aminotransferase family, MOCOS subfamily

Target Synonyms

MOCOS; Molybdenum cofactor sulfurase; MCS; MOS; MoCo sulfurase; hMCS; EC 2.8.1.9; Molybdenum cofactor sulfurtransferase

Target Background

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.