Identification of phytanoyl-CoA ligase as a distinct acyl-CoA ligase in peroxisomes from cultured human skin fibroblasts

FEBS Lett. 1993 May 10;322(2):101-4. doi: 10.1016/0014-5793(93)81546-c.


Phytanic acid accumulates in excessive amounts in Refsum disease, a rare neurological disorder, due to a defect in its alpha-oxidation enzyme system in peroxisomes. The activation of phytanic acid to phytanoyl-CoA by phytanoyl-CoA ligase is a prerequisite for its alpha-oxidation. The studies described in this manuscript report that phytanoyl-CoA ligase in peroxisomes is an enzyme distinct from the previously reported acyl-CoA ligases.

Publication types

  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Animals
  • Cells, Cultured
  • Coenzyme A Ligases / metabolism*
  • Epithelium / enzymology
  • Fibroblasts / enzymology
  • Humans
  • Liver / enzymology
  • Microbodies / enzymology*
  • Rats
  • Repressor Proteins*
  • Saccharomyces cerevisiae Proteins*
  • Skin / enzymology
  • Subcellular Fractions / enzymology


  • Repressor Proteins
  • Saccharomyces cerevisiae Proteins
  • Coenzyme A Ligases
  • lignoceroyl-CoA ligase
  • phytanoyl-CoA ligase
  • FAA2 protein, S cerevisiae
  • long-chain-fatty-acid-CoA ligase