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

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

Stable Identifier

R-HSA-389639

Type

Reaction [transition]

Species

Homo sapiens

Compartment

peroxisomal matrix

ReviewStatus

5/5

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

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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
16186124	Structure of human phytanoyl-CoA 2-hydroxylase identifies molecular mechanisms of Refsum disease Searls, T, McDonough, MA, Oppermann, U, Kavanagh, KL, Schofield, CJ, Butler, D	J Biol Chem	2005
11555634	Structure-function analysis of phytanoyl-CoA 2-hydroxylase mutations causing Refsum's disease Wierzbicki, AS, Kershaw, NJ, Mukherji, M, Chien, W, Clifton, IJ, Schofield, CJ, Lloyd, MD	Hum Mol Genet	2001