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首页> 外文期刊>Canadian Journal of Physiology and Pharmacology >Calcium and the damage pathways in muscular dystrophy.
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Calcium and the damage pathways in muscular dystrophy.

机译：钙和肌肉营养不良的损伤途径。

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摘要

Duchenne muscular dystrophy (DMD) is a severe muscle-wasting disease caused by the absence of the cytoskeletal protein dystrophin. Experiments on the mdx mouse, a model of DMD, have shown that mdx muscles are particularly susceptible to stretch-induced damage. In this review, we discuss evidence showing that a series of stretched contractions of mdx muscle fibres causes a prolonged increase in resting intracellular calcium concentration ([Ca2+]i). The rise in [Ca2+]i is caused by Ca2+ entry through a class of stretch-activated channels (SACNSC) for which one candidate gene is TRPC1. We review the evidence for activation of SACNSC in muscle by reactive oxygen species (ROS) and suggest that stretch-induced ROS production is part of the pathway that triggers increased channel activity. When the TRPC1 gene was transfected into C2 myoblasts, expression occurred throughout the cell. Only when the TRPC1 gene was coexpressed with caveolin-3 did the TRPC1 protein express in the membrane. When TRPC1 was expressed in the membrane, it could be activated by ROS to produce Ca2+ entry and this entry was inhibited by PP2, an inhibitor of src kinase. These results suggest that stretched contractions activate ROS production, which activates src kinase. Activity of this kinase causes opening of SACNSC and allows Ca2+ entry. This pathway appears to be a significant cause of muscle damage in DMD.
机译：杜兴氏肌营养不良症（DMD）是一种严重的肌肉萎缩性疾病，由缺乏细胞骨架蛋白的肌营养不良蛋白引起。在mdx老鼠（一种DMD模型）上进行的实验表明，mdx肌肉特别容易受到拉伸引起的损伤。在这篇综述中，我们讨论的证据显示，mdx肌纤维的一系列拉伸收缩会导致静息细胞内钙浓度（[Ca2 +] i）延长。 [Ca2 +] i的升高是由Ca2 +通过一类拉伸激活通道（SACNSC）进入引起的，该通道的一个候选基因是TRPC1。我们审查了活性氧（ROS）激活肌肉中SACNSC的证据，并建议拉伸诱导的ROS产生是触发通道活性增加的途径的一部分。当将TRPC1基因转染到C2成肌细胞中时，表达在整个细胞中发生。仅当TRPC1基因与小窝蛋白3共表达时，TRPC1蛋白才在膜中表达。当TRPC1在膜中表达时，可以被ROS激活以产生Ca2 +进入，而该进入被src激酶抑制剂PP2抑制。这些结果表明拉伸的收缩激活了ROS的产生，从而激活了src激酶。该激酶的活性导致SACNSC的开放并允许Ca2 +进入。该途径似乎是DMD中肌肉损伤的重要原因。

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