Intraorganellar localization of CoASH‐independent phytanic acid oxidation in human liver peroxisomes

FEBS Letters

Singh, Inderjit¹  Pahan, Kalipada¹ 

[1] Department of Pediatrics, Medical University of South Carolina, 171 Ashley Avenue, Charleston, SC 29425, USA

关键词: Peroxisome;    Phytanic acid;    Phytanoyl-CoA ligase;    Phytanic acid α-oxidation;   

DOI  :  10.1016/0014-5793(93)80395-B

学科分类：生物化学/生物物理

来源: John Wiley & Sons Ltd.

【 摘 要 】

In human tissues phytanic acid is α-oxidized to pristanic acid in peroxisomes. Studies of the intraorganellar site of α-oxidation of [1-¹⁴C]phytanic acid to pristanic acid in peroxisomes isolated from human liver demonstrate that phytanoyl-CoA ligase is present in the peroxisomal membrane and that the enzyme system for α-oxidation of phytanic acid to pristanic acid is in the peroxisomal matrix. In contrast to the β-oxidation system for fatty acids, the substrate for α-oxidation is free phytanic acid. The studies described in this manuscript report a novel fatty acid oxidation system where the substrate for the enzyme system is free fatty acid; however, phytanoyl-CoA ligase regulates the α-oxidation of phytanic acid at the organellar (peroxisomal) level.

【 授权许可】

Unknown