This gene encodes a mitochondrion outer membrane protein that catalyzes the hydroxylation of L-tryptophan metabolite, L-kynurenine, to form L-3-hydroxykynurenine. Studies in yeast identified this gene as a therapeutic target for Huntington disease. [provided by RefSeq, Oct 2011],
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Function:
Catalytic activity:L-kynurenine + NADPH + O(2) = 3-hydroxy-L-kynurenine + NADP(+) + H(2)O.,cofactor:FAD.,Function:Catalyzes the hydroxylation of L-kynurenine (L-Kyn) to form 3-hydroxy-L-kynurenine (L-3OHKyn). Required for synthesis of quinolinic acid, a neurotoxic NMDA receptor antagonist and potential endogenous inhibitor of NMDA receptor signaling in axonal targeting, synaptogenesis and apoptosis during brain development. Quinolinic acid may also affect NMDA receptor signaling in pancreatic beta cells, osteoblasts, myocardial cells, and the gastrointestinal tract.,miscellaneous:Increased in neuroinflammatory conditions. Inhibitors are investigated as potential neuroprotective drugs since they lead to an increased level of kynurenic acid, a neuroprotective NMDA receptor agonist.,pathway:Cofactor biosynthesis; NAD(+) biosynthesis; pyridine-2,3-dicarboxylate from L-kynurenine: step 1/3.,similarity:Belongs to the aromatic-ring hydroxylase family. KMO subfamily.,tissue specificity:Highest levels in placenta and liver. Detectable in kidney.,
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Cellular Localization:
Mitochondrion outer membrane ; Multi-pass membrane protein .
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Tissue Expression:
Highest levels in placenta and liver. Detectable in kidney.
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Research Areas:
>>Tryptophan metabolism ;
>>Metabolic pathways ;
>>Biosynthesis of cofactors