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单词 Mitochondrial myopathy
释义

Mitochondrial myopathy

中文百科

线粒体肌病

线粒体肌病是一种由线粒体的代谢缺陷脱引起的肌病,属于线粒体病。这种疾病也是一种母系遗传病,可根据临床不同症候群再细分为几种疾病。1962年,Luft首次采用改良戈莫理氏染色法(Gömöri trichrome stain,MGT)发现。在活体检查中,线粒体肌病患者的肌肉组织中常带有“破碎红纤维”(ragged red fibers),这些肌纤维内含有轻度累积的糖原和中性脂。此外,破红肌纤维中的琥珀酸脱氢酶活性增强,而细胞色素c氧化酶的活性则会减弱。

英语百科

Mitochondrial myopathy 线粒体肌病

Mitochondrial myopathies are types of myopathies associated with mitochondrial disease. On biopsy, the muscle tissue of patients with these diseases usually demonstrate "ragged red" muscle fibers. These ragged-red fibers contain mild accumulations of glycogen and neutral lipids, and may show an increased reactivity for succinate dehydrogenase and a decreased reactivity for cytochrome c oxidase. Inheritance is maternal (non-Mendelian extranuclear). There are several subcategories of mitochondrial myopathies.

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