慢性进行性眼外肌麻痹中线粒体细胞色素c氧化酶的“全或无”阳性：超微结构-细胞化学研究

"All-or-none" cytochrome c oxidase positivity in mitochondria in chronic progressive external ophthalmoplegia: an ultrastructural--cytochemical study.

作者信息

Matsuoka T, Goto Y, Nonaka I

机构信息

Division of Ultrastructural Research, National Center of Neurology and Psychiatry, Tokyo, Japan.

出版信息

Muscle Nerve. 1993 Feb;16(2):206-9. doi: 10.1002/mus.880160215.

DOI:10.1002/mus.880160215

PMID:8381519

Abstract

Teased single muscle fibers from 6 patients with chronic progressive external ophthalmoplegia (CPEO) showed a segmental defect in cytochrome c oxidase (COX) activity. On ultrastructural--cytochemical examination, the majority of mitochondria in COX-positive segments were COX-positive, whereas all mitochondria in COX-negative segments were COX-negative. This "all-or-none" COX positivity in mitochondria in CPEO with deleted mitochondrial DNA can be explained by the "threshold effect," which induces the tissue-specific involvement and clinical heterogeneity.

摘要

从6例慢性进行性眼外肌麻痹（CPEO）患者中分离出的单根肌纤维显示细胞色素c氧化酶（COX）活性存在节段性缺陷。在超微结构细胞化学检查中，COX阳性节段中的大多数线粒体为COX阳性，而COX阴性节段中的所有线粒体均为COX阴性。线粒体DNA缺失的CPEO中线粒体这种“全或无”的COX阳性可通过“阈值效应”来解释，该效应导致组织特异性受累和临床异质性。