MITOCHONDRIAL OXIDATION OF PHYTANIC ACID IN HUMAN AND MONKEY LIVER - IMPLICATION THAT REFSUMS DISEASE IS NOT A PEROXISOMAL DISORDER

被引：49

作者：

WATKINS, PA

MIHALIK, SJ

SKJELDAL, OH

机构：

[1] JOHNS HOPKINS UNIV,SCH MED,DEPT NEUROSURG,BALTIMORE,MD 21205

[2] JOHNS HOPKINS UNIV,SCH MED,DEPT PEDIAT,BALTIMORE,MD 21205

[3] UNIV OSLO,RIKSHOSP,INST CLIN BIOCHEM,OSLO,NORWAY

[4] UNIV OSLO,RIKSHOSP,DEPT PEDIAT,OSLO,NORWAY

来源：

BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS | 1990年 / 167卷 / 02期

关键词：

D O I：

10.1016/0006-291X(90)92064-7

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：

The subcellular site of oxidation of [1-14C]phytanic acid to 14CO2 was investigated in human and monkey liver. In both species, this activity was associated with fractions enriched in mitochondria. Fractions enriched in peroxisomes had no detectable phytanic acid oxidase activity. The mitochondrial inhibitors antimycin A and rotenone significantly decreased 14CO2 production in mitochondria-rich fractions from human and monkey liver. These inhibitors also blocked phytanic acid oxidation in cultured human skin fibroblasts. These data suggest that alpha-oxidation of phytanic acid is a mitochondrial rather than a peroxisomal process in primates. © 1990.

引用

页码：580 / 586

页数：7

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