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

Synonyms

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

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 McDonough, MA, Kavanagh, KL, Butler, D, Searls, T, Oppermann, U, Schofield, CJ	J Biol Chem	2005
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