Source:http://www.reactome.org/biopax/48887BiochemicalReaction4249
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Authored: D'Eustachio, P, 2009-01-10 20:36:15,
Edited: D'Eustachio, P, 2009-01-10 20:36:15,
Peroxisomal phytanoyl-CoA dioxygenase catalyzes the reaction of phytanoyl-CoA, 2-oxoglutarate, and O2 to form 2-hydroxyphytanoyl-CoA, succinate, and CO2. The mature form of the enzyme lacks the first 30 amino acid residues of the full-length polypeptide and is complexed with Fe++. Mutations in this enzyme are the commonest cause of Refsum disease (Mukherji et al. 2001; McDonough et al. 2005).,
Reviewed: Jassal, B, 2009-02-27 15:21:18
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phytanoyl-CoA + 2-oxoglutarate + O2 => 2-hydroxyphytanoyl-CoA + succinate + CO2
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1.14.11.18
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