Molybdenum Cofactor Sulfurase (MOCOS) Antibody – Rabbit Polyclonal

MOCOS: 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. Defects in MOCOS are the cause of xanthinuria type 2 (XU2). Xanthinuria is 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, patient suffering of XU2 cannot metabolize allopurinol into oxypurinol due to dual deficiency of xanthine dehydrogenase and aldehyde oxidase. Belongs to the class-V pyridoxal-phosphate-dependent aminotransferase family. MOCOS subfamily.

Protein type: EC 2.8.1.9; Lyase

Chromosomal Location of Human Ortholog: 18q12

Cellular Component: cytosol

Molecular Function: transferase activity; Mo-molybdopterin cofactor sulfurase activity; molybdenum ion binding; lyase activity; pyridoxal phosphate binding

Biological Process: vitamin metabolic process; Mo-molybdopterin cofactor biosynthetic process; molybdopterin cofactor biosynthetic process; water-soluble vitamin metabolic process; molybdopterin cofactor metabolic process