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一种伴有包被小泡和管状聚集物的慢性肌病。

A chronic myopathy with coated vesicles and tubular masses.

作者信息

Carpenter S, Karpati G, Holland P

机构信息

Department of Neurology-Neurosurgery, McGill University, Montreal, Quebec, Canada.

出版信息

Neuromuscul Disord. 1992;2(3):209-16. doi: 10.1016/0960-8966(92)90008-t.

Abstract

Two muscle biopsies from a 38-yr-old man with a lifelong mild chronic nonprogressive myopathy, showed accumulations of randomly oriented tubules of T-tubular origin, some of which had become greatly dilated and had accumulated osmiophilic material. The tubular masses lacked oxidative enzyme activity but during their evolution acquired esterase and acid phosphatase activity. Tubular areas appeared to break down and lead to intrusions of extracellular space into the center of fibers. Coated vesicles were numerous and often appeared to arise from the tubules. No abnormality could be detected on SDS PAGE electrophoresis of fractions from sucrose gradients.

摘要

一名38岁患有终生轻度慢性非进行性肌病的男性的两份肌肉活检显示,有源自T小管的随机排列的小管聚集,其中一些已显著扩张并积累了嗜锇物质。这些管状团块缺乏氧化酶活性,但在其演变过程中获得了酯酶和酸性磷酸酶活性。管状区域似乎分解,导致细胞外空间侵入纤维中心。有被小泡数量众多,且常常似乎源自这些小管。在蔗糖梯度分级分离物的SDS - PAGE电泳上未检测到异常。

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