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MOCOS sulfurates the molybdenum cofactor of xanthine oxidase (XDH (show XDH Proteins)) and aldehyde oxidase 1 (AOX1 (show AOX1 Proteins)), which is required for their activities.
Two mutations, both in the N-terminal domain of the Human Molybdenum Cofactor Sulfurase (HMCS), were reported in patients with type II xanthinuria.
MOCOS loss of function variant is highly likely to cause the renal syndrome in the affected animals
MOCOS sulfurates the molybdenum cofactor of xanthine dehydrogenase (XDH\; MIM 607633) and aldehyde oxidase (AOX1\; MIM 602841), which is required for their enzymatic activities (Ichida et al., 2001
, molybdenum cofactor sulfurtransferase
, molybdenum cofactor sulfurase
, protein organdy
, Molybdenum cofactor sulfurase-like