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肌营养不良蛋白作为骨骼肌中的一种机械化学转换器。

Dystrophin as a mechanochemical transducer in skeletal muscle.

作者信息

Brown S C, Lucy J A

机构信息

Department of Biochemistry and Chemistry, Royal Free Hospital School of Medicine, London, UK.

出版信息

Bioessays. 1993 Jun;15(6):413-9. doi: 10.1002/bies.950150608.

Abstract

This review is primarily concerned with two key issues in research on dystrophin: (1) how the protein interacts with the plasma membrane of skeletal muscle fibres and (2) how an absence of dystrophin gives rise to Duchenne muscular dystrophy. In relation to the first point, we suggest that the post-translational acylation of dystrophin may contribute to its interaction with the plasma membrane. Regarding the second point, it is generally considered that an absence of dystrophin makes the plasma membrane susceptible to damage by contraction/relaxation cycles. In this connection, we propose that the progressive nature of Duchenne dystrophy, and the phenotypic characteristics of mdx mice, are more consistent with dystrophin functioning as a mechanical transducer that transmits growth stimuli from the enlarging skeleton to the muscle. On the basis of this hypothesis, dystrophin-deficient muscles would be unable to grow at the same rate as the skeleton.

摘要

本综述主要关注肌营养不良蛋白研究中的两个关键问题

(1)该蛋白如何与骨骼肌纤维的质膜相互作用;(2)肌营养不良蛋白的缺失如何导致杜兴氏肌营养不良症。关于第一点,我们认为肌营养不良蛋白的翻译后酰化可能有助于其与质膜的相互作用。关于第二点,一般认为肌营养不良蛋白的缺失会使质膜易受收缩/舒张循环的损伤。在这方面,我们提出,杜兴氏肌营养不良症的进行性本质以及mdx小鼠的表型特征,与肌营养不良蛋白作为一种将生长刺激从不断增大的骨骼传递到肌肉的机械转导器的功能更为一致。基于这一假设,缺乏肌营养不良蛋白的肌肉将无法以与骨骼相同的速度生长。

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