Reaction: phytanoyl-CoA + 2-oxoglutarate + O2 => 2-hydroxyphytanoyl-CoA + succinate + CO2
- in pathway: Alpha-oxidation of phytanate
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).
Reaction - small molecule participants:
3S2HPhy-CoA [peroxisomal matrix]
SUCCA [peroxisomal matrix]
CO2 [peroxisomal matrix]
O2 [peroxisomal matrix]
Phytanoyl-CoA [peroxisomal matrix]
2OG [peroxisomal matrix]
Reactome.org reaction link: R-HSA-389639
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Reaction input - small molecules:
dioxygen
phytanoyl-CoA
2-oxoglutarate(2-)
Reaction output - small molecules:
2-hydroxyphytanoyl-CoA
succinate(2-)
carbon dioxide
Reactome.org link: R-HSA-389639