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Identification of phytanoyl‐CoA ligase as a distinct acyl‐CoA ligase in peroxisomes from cultured human skin fibroblasts
Author(s) -
Pahan Kalipada,
Cofer Joseph,
Baliga Prabhakar,
Singh Inderjit
Publication year - 1993
Publication title -
febs letters
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.593
H-Index - 257
eISSN - 1873-3468
pISSN - 0014-5793
DOI - 10.1016/0014-5793(93)81546-c
Subject(s) - phytanic acid , dna ligase , peroxisome , ubiquitin ligase , biochemistry , enzyme , acyl coa , microbody , biology , chemistry , ubiquitin , gene
Phytanic acid accumulates in excessive amounts in Refsum disease, a rare neurological disorder, due to a defect in its α‐oxidation enzyme system in peroxisomes. The activation of phytanic acid to phytanoyl‐CoA by phytanoyl‐CoA ligase is a prerequisite for its α‐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.