INTRAORGANELLAR LOCALIZATION OF COASH-INDEPENDENT PHYTANIC ACID OXIDATION IN HUMAN LIVER PEROXISOMES

被引:22
作者
PAHAN, K [1 ]
SINGH, I [1 ]
机构
[1] MED UNIV S CAROLINA,DEPT PEDIAT,171 ASHLEY AVE,CHARLESTON,SC 29425
关键词
PEROXISOME; PHYTANIC ACID; PHYTANOYL-COA LIGASE; PHYTANIC ACID ALPHA-OXIDATION;
D O I
10.1016/0014-5793(93)80395-B
中图分类号
Q5 [生物化学]; Q7 [分子生物学];
学科分类号
071010 ; 081704 ;
摘要
In human tissues phytanic acid is alpha-oxidized to pristanic acid in peroxisomes. Studies of the intraorganellar site of alpha-oxidation of [1-C-14]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 alpha-oxidation of phytanic acid to pristanic acid is in the peroxisomal matrix. In contrast to the beta-oxidation system for fatty acids, the substrate for alpha-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 alpha-oxidation of phytanic acid at the organellar (peroxisomal) level.
引用
收藏
页码:154 / 158
页数:5
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