线粒体脑肌病中的肌肉辅酶Q10。

Muscle coenzyme Q10 in mitochondrial encephalomyopathies.

作者信息

Matsuoka T, Maeda H, Goto Y, Nonaka I

机构信息

Division of Ultrastructural Research, National Institute of Neuroscience, National Center of Neurology and Psychiatry (NCNP), Tokyo, Japan.

出版信息

Neuromuscul Disord. 1991;1(6):443-7. doi: 10.1016/0960-8966(91)90007-f.

DOI:10.1016/0960-8966(91)90007-f

PMID:1822356

Abstract

Coenzyme Q10 (CoQ) content was measured in isolated muscle mitochondria from 25 patients with mitochondrial encephalomyopathies (MEM), most of whom had mitochondrial DNA mutations. The CoQ level was significantly lower in MEM patients than in controls. CoQ levels varied widely from patient to patient, especially in those with chronic progressive external ophthalmoplegia including Kearns-Sayre syndrome, which may explain, at least in part, the variable response of patients to CoQ administration.

摘要

在25例线粒体脑肌病（MEM）患者的分离肌肉线粒体中测量了辅酶Q10（CoQ）含量，其中大多数患者存在线粒体DNA突变。MEM患者的CoQ水平显著低于对照组。患者之间的CoQ水平差异很大，尤其是在患有慢性进行性眼外肌麻痹包括卡恩斯-塞尔综合征的患者中，这可能至少部分解释了患者对CoQ给药的不同反应。