Mitochondrial changes in skeletal muscle in amyotrophic lateral sclerosis and other neurogenic atrophies
Mitochondrial changes in skeletal muscle in amyotrophic lateral sclerosis and other neurogenic atrophies
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DOI：
10.1093/brain/awh540
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发表时间：
2005-08-01
期刊：
影响因子：
14.5
通讯作者：
Zierz, S
中科院分区：
文献类型：
--
作者：
Krasnianski, A;Deschauer, M;Zierz, S
以往的研究结果提示肌萎缩侧索硬化症(ALS)患者骨骼肌中存在特异性线粒体功能障碍。对24例散发性ALS患者肌肉组织中线粒体标志物活性的组织化学分布、线粒体(Mt)/核(N)DNA比值、柠檬酸合成酶(CS)和呼吸链酶活性进行了研究。将这些数据与23例其他神经源性萎缩(NAS)患者和21名健康对照进行比较。肌肉组织学显示，两组患者肌肉纤维中线粒体氧化活性的局灶性减弱的迹象相似。ALS和NA患者的mt/nDNA比值与健康对照组相比仅有轻微下降。ALS和NA患者的线粒体标志物CS和琥珀酸脱氢酶比活性均显著升高。呼吸链酶比活力在三组间均无显著差异。结论：肌肉的组织化学、生化和分子线粒体改变不是ALS所特有的，而是伴随着其他NAS的改变。
Previous findings suggested specific mitochondrial dysfunction in skeletal muscle of patients with amyotrophic lateral sclerosis (ALS). To answer the question of whether the dysfunction is specific, we investigated the histochemical distribution of mitochondrial marker activities, the ratio of mitochondrial (mt) versus nuclear (n) DNA, and the activities of citrate synthase (CS) and respiratory chain enzymes in muscle biopsies of 24 patients with sporadic ALS. The data were compared with those in 23 patients with other neurogenic atrophies (NAs), and 21 healthy controls. Muscle histology revealed similar signs of focally diminished mitochondrial oxidation activity in muscle fibres in both diseased groups. There was only minimal decline of mt/nDNA ratios in ALS and NA patients in comparison with healthy controls. The specific activities of mitochondrial markers CS and succinate dehydrogenase were significantly increased in both ALS and NA patients. The specific activities of respiratory chain enzymes were not significantly different in all three groups. It is concluded that the histochemical, biochemical and molecular mitochondrial changes in muscle are not specific for ALS, but accompany other NAs as well.