膜通道和肌肉疾病。

Australian paediatric journal Pub Date : 1988-01-01
A H Bretag
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引用次数: 0

摘要

本文综述了迄今为止在单通道水平上表征的膜通道及其在肌肉功能障碍中的可能参与。利用Torbergsen综合征患者的离体外肋间肌和化学诱导肌强直的离体大鼠肌肉进行的实验表明,膜通道在肌肉疾病中的进一步参与。异常通道的存在和正常通道的缺失都可以赋予肌层和肌浆网状膜特性,从而导致肌肉行为的特殊和独特缺陷。
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Membrane channels and muscle disease.

Membrane channels so far characterized at the single channel level and their possible involvement in muscle dysfunction are reviewed. Experiments using isolated external intercostal muscle from a patient with Torbergsen syndrome and isolated rat muscles with chemically induced myotonia suggest further involvement of membrane channels in muscle disease. Both the presence of unusual channels and the absence of normal channels can confer properties on the sarcolemmal and sarcoplasmic reticular membranes which give rise to peculiar and distinctive defects in muscle behaviour.

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