植烷酸在人和猴肝脏中的线粒体氧化：提示雷夫叙姆病并非过氧化物酶体疾病。

Mitochondrial oxidation of phytanic acid in human and monkey liver: implication that Refsum's disease is not a peroxisomal disorder.

作者信息

Watkins P A, Mihalik S J

机构信息

Kennedy Institute, Baltimore, MD.

出版信息

Biochem Biophys Res Commun. 1990 Mar 16;167(2):580-6. doi: 10.1016/0006-291x(90)92064-7.

DOI:10.1016/0006-291x(90)92064-7

PMID:1690986

Abstract

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.

摘要

在人和猴的肝脏中研究了[1-¹⁴C]植烷酸氧化为¹⁴CO₂的亚细胞位点。在这两个物种中，该活性与富含线粒体的组分相关。富含过氧化物酶体的组分没有可检测到的植烷酸氧化酶活性。线粒体抑制剂抗霉素A和鱼藤酮显著降低了来自人和猴肝脏的富含线粒体的组分中¹⁴CO₂的产生。这些抑制剂也阻断了培养的人皮肤成纤维细胞中的植烷酸氧化。这些数据表明，在灵长类动物中，植烷酸的α-氧化是一个线粒体过程而非过氧化物酶体过程。