A saturated 20-carbon branched-chain fatty acid which can only be derived from dietary sources. Under normal conditions, phytanic acid is degraded via α-oxidation (oxidative decarboxylation) to produce pristanic acid, which then undergoes β-oxidation as part of the metabolism process. Degradation of phytanic acid is impaired in patients with peroxisomal disorders or diseases such as infantile phytanic acid storage disease or Refsum's disease.
1 Watkins, P.A., Howard, A.E., Mihalik, S.J. Phytanic acid must be activated to phytanoyl-CoA prior to its a-oxidation in rat liver peroxisomes. Biochem Biophys Acta 1214 288-294 (1994). 2 Watkins, P.A., Howard, A.E., Gould, S.J., et al. Phytanic acid activation in rat liver peroxisomes is catalyzed by long-chain acyl-CoA synthetase. J Lipid Res 37 2288-2295 (1996). 3 Wanders, R.J.A., Boltshauser, E., Steinmann, B., et al. Infantile phytanic acid storage disease, a disorder of peroxisome biogenesis: A case report. J Neurol Sci 98 1-11 (1990).
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