...
首页> 外文期刊>American Journal of Physiology >Can't live with or without it: calcium and its role in Duchenne muscular dystrophy-induced muscle weakness. Focus on 'SERCAl overexpression minimizes skeletal muscle damage in dystrophic mouse models'
【24h】

Can't live with or without it: calcium and its role in Duchenne muscular dystrophy-induced muscle weakness. Focus on 'SERCAl overexpression minimizes skeletal muscle damage in dystrophic mouse models'

机译:不能忍受或没有它:钙及其在杜南肌营养不良症诱导的肌肉弱点中的作用。 专注于“血清过表达,最大限度地减少营养不良小鼠模型中的骨骼肌损伤”

获取原文
获取原文并翻译 | 示例
           

摘要

duchenne muscular dystrophy (DMD) is an X-linked genetic disorder caused by the lack of the membrane-associated protein, dystrophin, which leads to premature death (5). The absence of dystrophin increases susceptibility of fibers to muscle damage. Repeated myofiber damage results in ineffective muscle repair and development of pseudo-hypertrophied muscles that are weak for their given size and mass. As of yet, the mechanisms underlying the functional impairment in dystrophic muscle have not been completely elucidated. However, recent evidence points to elevated intracellular Ca2+ homeo-stasis being a cause or facilitator to the development of muscle weakness in muscular dystrophies (1, 4, 8, 9).Ca2+ is the major regulator of muscle force generation in skeletal muscle. At the muscle level, an action potential propagating along the sarcolemma traverses down the transverse tubules where the voltage-sensing dihydropyridine receptor mechanically interacts with ryanodine receptor 1 (RyRl) to release Ca2+ from its main storage site, the sarcoplasmic reticulum (SR).
机译:Duchenne肌营养不良(DMD)是一种由缺乏膜相关蛋白,营养不良蛋白引起的X链接遗传疾病,导致过早死亡(5)。没有营养不良蛋白增加纤维对肌肉损伤的敏感性。重复的肌电纤维损伤导致无效的肌肉修复和开发伪肥大肌肉,对其给定的大小和质量薄弱。据呢,营养不良肌肉功能损伤的机制尚未完全阐明。然而,最近的证据表明升高的细胞内Ca2 + Homeo-stasis是肌营养不良(1,4,8,9).ca2 +是肌肉肌肉肌肉肌肉肌肉发电的主要调节因子的原因或促进者。在肌肉水平处,沿着Sarcolemma传播的动作电位遍历横向小管,其中电压传感二氢吡啶受体机械地与ryanodine受体1(Ryrl)与其主储存部位释放Ca2 +,肌肉网状物(SR)。

著录项

相似文献

  • 外文文献
  • 中文文献
  • 专利
获取原文

客服邮箱:kefu@zhangqiaokeyan.com

京公网安备:11010802029741号 ICP备案号:京ICP备15016152号-6 六维联合信息科技 (北京) 有限公司©版权所有
  • 客服微信

  • 服务号