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. Note: This description may include information from UniProtKB.
Protein type: EC 1.17.1.4; EC 1.17.3.2; Nucleotide Metabolism - purine; Oxidoreductase; Secondary Metabolites Metabolism - caffeine; Xenobiotic Metabolism - drug metabolism - other enzymes
Chromosomal Location of Human Ortholog: 17 E2|17 45.25 cM
Cellular Component:  cytoplasm; cytosol; extracellular region; extracellular space; peroxisome; sarcoplasmic reticulum
Molecular Function:  2 iron, 2 sulfur cluster binding; electron transfer activity; FAD binding; flavin adenine dinucleotide binding; iron ion binding; iron-sulfur cluster binding; metal ion binding; molybdenum ion binding; molybdopterin cofactor binding; nitrite reductase (NO-forming) activity; oxidoreductase activity; protein binding; protein homodimerization activity; xanthine dehydrogenase activity; xanthine oxidase activity
Biological Process:  activation of cysteine-type endopeptidase activity involved in apoptotic process; hypoxanthine oxidation; lactation; negative regulation of endothelial cell differentiation; negative regulation of endothelial cell proliferation; negative regulation of gene expression; negative regulation of protein kinase B signaling; negative regulation of protein phosphorylation; negative regulation of vascular endothelial growth factor signaling pathway; negative regulation of vasculogenesis; oxidation-reduction process; positive regulation of p38MAPK cascade; positive regulation of reactive oxygen species metabolic process; regulation of epithelial cell differentiation; response to aluminum ion; xanthine catabolic process
Reference #:  Q00519 (UniProtKB)
Alt. Names/Synonyms: Xanthine dehydrogenase; Xanthine dehydrogenase/oxidase; Xanthine oxidase; Xanthine oxidoreductase; XD; Xdh; XO; Xor; Xox-1; Xox1
Gene Symbols: Xdh
Molecular weight: 146,562 Da
Basal Isoelectric point: 7.62  Predict pI for various phosphorylation states
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XDH

Protein Structure Not Found.


Cross-references to other databases:  STRING  |  Reactome  |  BioGPS  |  Pfam  |  ENZYME  |  Phospho.ELM  |  NetworKIN  |  UniProtKB  |  Entrez-Gene  |  Ensembl Gene