phytanoyl-CoA + 2-oxoglutarate + O2 => 2-hydroxyphytanoyl-CoA + succinate + CO2

Stable Identifier
R-HSA-389639
Type
Reaction [transition]
Species
Homo sapiens
Compartment
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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).

Literature References
PubMed ID Title Journal Year
11555634 Structure-function analysis of phytanoyl-CoA 2-hydroxylase mutations causing Refsum's disease

Mukherji, M, Chien, W, Kershaw, NJ, Clifton, IJ, Schofield, CJ, Wierzbicki, AS, Lloyd, MD

Hum Mol Genet 2001
16186124 Structure of human phytanoyl-CoA 2-hydroxylase identifies molecular mechanisms of Refsum disease

McDonough, MA, Kavanagh, KL, Butler, D, Searls, T, Oppermann, U, Schofield, CJ

J Biol Chem 2005
Participants
Participant Of
Catalyst Activity
Catalyst Activity
Title
phytanoyl-CoA dioxygenase activity of PHYH:Fe++ [peroxisomal matrix]
Physical Entity
Activity
Orthologous Events
Cross References
Rhea
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Reviewed
Created