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

FEBS Letters

Baliga, Prabhakar²  Singh, Inderjit¹  Pahan, Kalipada¹  Cofer, Joseph² 

[1] Department of Pediatrics and Surgery, Division of Developmental Neurogenetics, Medical University of South Carolina, Charleston, SC 29425, USA;Department of Surgery, Division of Developmental Neurogenetics, Medical University of South Carolina, Charleston, SC 29425, USA

关键词: Phytanoyl-CoA ligase;    Peroxisome;    Phytanic acid;    Human skin fibroblast;   

DOI  :  10.1016/0014-5793(93)81546-C

学科分类：生物化学/生物物理

来源: John Wiley & Sons Ltd.

【 摘 要 】

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.

【 授权许可】

Unknown