Human Molybdenum cofactor sulfurase ELISA Kit | MOCOS elisa kit
Human Molybdenum cofactor sulfurase ELISA Kit
NCBI and Uniprot Product Information
NCBI Description
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 Description
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: 18q12.2
Cellular Component: cytosol
Molecular Function: Mo-molybdopterin cofactor sulfurase activity; protein binding
Biological Process: molybdopterin cofactor biosynthetic process; molybdopterin cofactor metabolic process
Disease: Xanthinuria, Type Ii
Research Articles on MOCOS
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Product Notes
The Human MOCOS mocos (Catalog #AAA9427066) is an ELISA Kit and is intended for research purposes only. The product is available for immediate purchase. The AAA9427066 ELISA Kit recognizes Human MOCOS. It is sometimes possible for the material contained within the vial of "Molybdenum cofactor sulfurase, ELISA Kit" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
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