Cytochrome c oxidase deficient muscle fibres:: Substantial variation in their proportions within skeletal muscles from patients with mitochondrial myopathy

被引：14

作者：

Barron, MJ

Chinnery, PF

Howel, D

Blakely, EL

Schaefer, AM

Taylor, RW

Turnbull, DM ^{[1
]}

机构：

[1] Univ Newcastle Upon Tyne, Sch Neurol Neurosci & Psychiat, Mitochondrial Res Grp, Newcastle Upon Tyne NE2 4HH, Tyne & Wear, England

[2] Univ Newcastle Upon Tyne, Sch Populat & Hlth Sci, Newcastle Upon Tyne NE2 4HH, Tyne & Wear, England

来源：

NEUROMUSCULAR DISORDERS | 2005年 / 15卷 / 11期

基金：

英国惠康基金;

关键词：

D O I：

10.1016/j.nmd.2005.06.018

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

Mitochondrial DNA (mtDNA) disease is a common cause of myopathy and the presence of histochemically demonstrated cytochrome c oxidase (COX) deficiency is an extremely useful diagnostic feature. However, there is currently no quantitative information regarding the variability of COX deficiency within or between muscles. This study addresses this issue by studying a number of skeletal muscle samples obtained at post-mortem from three patients with mitochondrial disease due to established mitochondrial DNA defects. COX deficient muscle fibres were enumerated in sections of these muscles and analysed according to patient, individual muscle, position within a particular muscle and sample size. Descriptive statistics were generated followed by an analysis of variance (ANOVA) to assess the effect of these parameters on the mean percentage of COX deficient fibres. We observed statistically significant variation in the percentage of COX deficient fibres within individual muscles from each patient for samples sizes of between 100 and 400 fibres. Our results have implications for the way in which biopsies of skeletal muscle are used for the assessment of disease severity, progression and response to treatment. (C) 2005 Elsevier B.V. All rights reserved.

引用

页码：768 / 774

页数：7

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