Studies on phytanic acid alpha-oxidation in rat liver and cultured human skin fibroblasts

Biochim Biophys Acta. 1993 Apr 23;1167(3):345-50. doi: 10.1016/0005-2760(93)90239-6.


We have studied the alpha-oxidation of phytanic acid in rat liver and human skin fibroblasts in order to try to resolve the controversial issue of the subcellular site of alpha-oxidation of phytanic acid. The results show that isolated mitochondria are able to alpha-oxidize phytanic acid whereas isolated peroxisomes show no phytanic acid alpha-oxidation activity. Intact hepatocytes were found to alpha-oxidize phytanic acid at a rate which is more than 20-fold higher than the activity found in postnuclear supernatant fractions incubated under optimal conditions. The alpha-oxidation of phytanic acid was found to be sensitive to inhibitors of the respiratory chain and an uncoupler of oxidative phosphorylation. Furthermore, the alpha-oxidation of phytanic acid was found to be deficient in cultured human skin fibroblasts with an inherited deficiency of cytochrome c oxidase and in fibroblasts with a deficiency of functional peroxisomes. We conclude that mitochondria are indispensable for phytanic acid alpha-oxidation. Furthermore, we propose that one (or more) of the partial reactions in phytanic acid alpha-oxidation proceeds in peroxisomes leading to the concept that phytanic acid oxidation in the intact cell requires the participation of both mitochondria and peroxisomes.

MeSH terms

  • Adenosine Triphosphate / metabolism
  • Animals
  • Cells, Cultured / metabolism
  • Cytochrome-c Oxidase Deficiency
  • Fibroblasts / metabolism
  • Humans
  • Liver / metabolism*
  • Microbodies / metabolism
  • Mitochondria / metabolism
  • Mitochondria, Liver / metabolism
  • Oxidation-Reduction
  • Oxidative Phosphorylation / drug effects
  • Phytanic Acid / metabolism*
  • Rats
  • Skin / metabolism*


  • Phytanic Acid
  • Adenosine Triphosphate