Identification of phytanoyl-CoA ligase as a distinct acyl-CoA ligase in peroxisomes from cultured human skin fibroblasts.
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.
Pahan
K
K
Department of Pediatrics, Medical University of South Carolina, Charleston 29425.
Cofer
J
J
Baliga
P
P
Singh
I
I
D000818:Animals; D002478:Cells, Cultured; D003066:Coenzyme A Ligases / Q000378:metabolism*; D004848:Epithelium / Q000201:enzymology; D005347:Fibroblasts / Q000201:enzymology; D006801:Humans; D008099:Liver / Q000201:enzymology; D008830:Microbodies / Q000201:enzymology*; D051381:Rats; D012097:Repressor Proteins; D029701:Saccharomyces cerevisiae Proteins; D012867:Skin / Q000201:enzymology; D013347:Subcellular Fractions / Q000201:enzymology; D000098859:Long-Chain-Fatty-Acid-CoA Ligase
D012097:Repressor Proteins; D029701:Saccharomyces cerevisiae Proteins; D003066:Coenzyme A Ligases; C044125:lignoceroyl-CoA ligase; C080977:phytanoyl-CoA ligase; C090799:FAA2 protein, S cerevisiae; D000098859:Long-Chain-Fatty-Acid-CoA Ligase