phytanoyl-Coenzyme A (triethylammonium salt)

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.

参考文献

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).