Abstract
Phytanic acid is a 3-methyl branched fatty acid that cannot be degraded by P-oxidation. Instead, one carbon atom is removed to give pristanic acid by a single round of peroxisomal a-oxidation. Phytanoyl-CoA 2- hydroxylase (PAHX), an iron(II) and 2-oxoglutarate oxygenase, catalyses the first step in this pathway. Defects in PAHX cause some forms of adult Refsum’s disease, in which phytanic acid accumulates (Wanders et al., 2001; Wierzbicki et al., 2002).
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