Identification of phytanoyl‐CoA ligase as a distinct acyl‐CoA ligase in peroxisomes from cultured human skin fibroblasts
- 10 May 1993
- journal article
- Published by Wiley in FEBS Letters
- Vol. 322 (2) , 101-104
- https://doi.org/10.1016/0014-5793(93)81546-c
Abstract
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.Keywords
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