Xanthine Dehydrogenase/Oxidase (XDH), Partial (XDH) Human Recombinant Protein

Xanthine dehydrogenase belongs to the group of molybdenum-containing hydroxylases involved in the oxidative metabolism of purines. The encoded protein has been identified as a moonlighting protein based on its ability to perform mechanistically distinct functions. Xanthine dehydrogenase can be converted to xanthine oxidase by reversible sulfhydryl oxidation or by irreversible proteolytic modification. Defects in xanthine dehydrogenase cause xanthinuria, may contribute to adult respiratory stress syndrome, and may potentiate influenza infection through an oxygen metabolite-dependent mechanism. [provided by RefSeq, Jan 2014]

XDH: Key enzyme in purine degradation. Catalyzes the oxidation of hypoxanthine to xanthine. Catalyzes the oxidation of xanthine to uric acid. Contributes to the generation of reactive oxygen species. Has also low oxidase activity towards aldehydes (in vitro). Defects in XDH are the cause of xanthinuria type 1 (XU1). 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. XU1 is due to isolated xanthine dehydrogenase. XU1 patients can metabolize allopurinol. Belongs to the xanthine dehydrogenase family.

Protein type: Oxidoreductase; EC 1.17.1.4; Xenobiotic Metabolism - drug metabolism - other enzymes; EC 1.17.3.2; Secondary Metabolites Metabolism - caffeine; Nucleotide Metabolism - purine

Chromosomal Location of Human Ortholog: 2p23.1

Cellular Component: extracellular space; peroxisome; cytosol

Molecular Function: molybdopterin cofactor binding; 2 iron, 2 sulfur cluster binding; UDP-N-acetylmuramate dehydrogenase activity; oxidoreductase activity, acting on the aldehyde or oxo group of donors; protein homodimerization activity; electron carrier activity; FAD binding; iron ion binding; xanthine dehydrogenase activity; xanthine oxidase activity

Biological Process: lactation; caspase activation; xanthine catabolic process; negative regulation of protein kinase B signaling cascade; negative regulation of protein amino acid phosphorylation; negative regulation of endothelial cell differentiation; nucleobase, nucleoside and nucleotide metabolic process; negative regulation of endothelial cell proliferation; purine nucleotide catabolic process; purine base metabolic process

Disease: Xanthinuria, Type I