Histological and biochemical assessment of mitochondrial function in dermatomyositis.

Ò. Miró, J. Casademont, J. Grau, D. Jarreta, A. Urbano-Márquez, F. Cardellach
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引用次数: 28

Abstract

OBJECTIVE Mitochondrial dysfunction in idiopathic inflammatory myopathies (IIM) remains a controversial issue. The aim of the present study was to investigate the correlation between histological abnormalities and the biochemical function of the skeletal muscle mitochondria from patients with dermatomyositis (DM). METHOD We evaluated 10 patients with a new diagnosis of DM and 15 healthy individuals, matched by age and gender. Muscle biopsy was routinely processed for histochemical studies and biochemical analysis of pure mitochondria. The percentages of ragged-red fibres (RRF), cytochrome c oxidase (COX)-negative fibres and succinic dehydrogenase (SDH) hyper-reactive fibres were calculated, oxygen utilization using different substrates was assessed polarographically, and enzymatic activity of individual complexes of the electron transport chain (ETC) and ATPase was measured spectrophotometrically. RESULTS We found an increased percentage of COX-negative and SDH hyper-reactive fibres in DM patients (0.82 and 1.82%, respectively) compared to controls (0.26 and 0.22%; P < 0.05 and P = 0.001, respectively); however, oxidation rates of different substrates and enzymatic activities of ETC and ATPase did not differ significantly between both groups. CONCLUSION The overall function of ETC from skeletal muscle mitochondria is not affected in DM.
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皮肌炎患者线粒体功能的组织学和生化评价。
目的特发性炎性肌病(IIM)的线粒体功能障碍仍然是一个有争议的问题。本研究旨在探讨皮肌炎(DM)患者骨骼肌线粒体的组织学异常与生化功能之间的相关性。方法我们评估了10例新诊断为糖尿病的患者和15例按年龄和性别匹配的健康人。肌肉活检常规处理纯线粒体的组织化学研究和生化分析。计算了粗红纤维(RRF)、细胞色素c氧化酶(COX)阴性纤维和琥珀酸脱氢酶(SDH)高反应纤维的百分比,极谱法评估了不同底物对氧的利用,分光光度法测定了电子传递链(ETC)和atp酶的单个配合物的酶活性。结果我们发现,与对照组(0.26%和0.22%)相比,DM患者中cox -阴性和SDH高反应纤维的比例(分别为0.82%和1.82%)有所增加;P < 0.05, P = 0.001);不同底物的氧化速率和ETC、atp酶活性在两组间无显著差异。结论糖尿病不影响骨骼肌线粒体ETC的整体功能。
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