Phytanoyl-CoA dioxygenase, peroxisomal

Definition:

Catalyzes the 2-hydroxylation of not only racemic phytanoyl-CoA and the isomers of 3-methylhexadecanoyl-CoA, but also a variety of other mono-branched 3-methylacyl-CoA esters (with a chain length of at least seven carbon atoms) and straight-chain acyl-CoA esters (with a chain length longer than four carbon atoms). Does not hydroxylate long and very long straight chain acyl-CoAs or 2-methyl- and 4-methyl-branched acyl-CoAs.

References:

[1]. S J Mihalik, et al. Identification of PAHX, a Refsum disease gene. Nat Genet. 1997 Oct;17(2):185-9. [Content Brief]

[2]. Veerle Foulon, et al. Further studies on the substrate spectrum of phytanoyl-CoA hydroxylase: implications for Refsum disease?. J Lipid Res. 2003 Dec;44(12):2349-55. [Content Brief]

[3]. Mridul Mukherji, et al. Utilization of sterol carrier protein-2 by phytanoyl-CoA 2-hydroxylase in the peroxisomal alpha oxidation of phytanic acid. Chem Biol. 2002 May;9(5):597-605. [Content Brief]

[4]. K Croes, et al. Phytanoyl-CoA hydroxylase: recognition of 3-methyl-branched acyl-coAs and requirement for GTP or ATP and Mg(2+) in addition to its known hydroxylation cofactors. J Lipid Res. 2000 Apr;41(4):629-36. [Content Brief]

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