The role of the dystrophin glycoprotein complex in muscle cell mechanotransduction.
The role of the dystrophin glycoprotein complex in muscle cell mechanotransduction.
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DOI：
10.1038/s42003-022-03980-y
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发表时间：
2022-09-27
影响因子：
5.9
通讯作者：
中科院分区：
文献类型：
--
作者：
肌营养不良蛋白是骨骼肌和心肌细胞中肌营养不良蛋白-糖蛋白复合物（DGC）的中心蛋白。肌营养不良蛋白将肌动蛋白细胞骨架连接到细胞外基质（ECM）。切断ECM和细胞内细胞骨架之间的联系对骨骼肌细胞的稳态具有破坏性影响，导致一系列肌营养不良症。此外，功能性DGC的丧失导致进行性扩张型心肌病和过早死亡。肌营养不良蛋白作为分子弹簧发挥作用，DGC在维持肌膜完整性方面起关键作用。此外，证据正在积累，将DGC与机械信号联系起来，尽管这种作用仍然不太清楚。这篇综述文章的目的是提供一个最新的观点DGC及其在机械转导的作用。我们首先讨论了肌肉细胞力学和功能之间的复杂关系，然后研究肌营养不良蛋白糖蛋白复合物在机械转导和维持肌肉细胞生物力学完整性中的作用。最后，我们回顾了当前的文献，以绘制出DGC信号传导如何与机械信号传导途径相交，以突出未来潜在的干预点，特别是重点关注心肌病。综述了营养不良糖蛋白复合物（DGC）在机械信号传导中的功能，概述了DGC的各种成分和潜在的机械致病机制，特别是当它们与肌营养不良症相关时。
Dystrophin is the central protein of the dystrophin-glycoprotein complex (DGC) in skeletal and heart muscle cells. Dystrophin connects the actin cytoskeleton to the extracellular matrix (ECM). Severing the link between the ECM and the intracellular cytoskeleton has a devastating impact on the homeostasis of skeletal muscle cells, leading to a range of muscular dystrophies. In addition, the loss of a functional DGC leads to progressive dilated cardiomyopathy and premature death. Dystrophin functions as a molecular spring and the DGC plays a critical role in maintaining the integrity of the sarcolemma. Additionally, evidence is accumulating, linking the DGC to mechanosignalling, albeit this role is still less understood. This review article aims at providing an up-to-date perspective on the DGC and its role in mechanotransduction. We first discuss the intricate relationship between muscle cell mechanics and function, before examining the recent research for a role of the dystrophin glycoprotein complex in mechanotransduction and maintaining the biomechanical integrity of muscle cells. Finally, we review the current literature to map out how DGC signalling intersects with mechanical signalling pathways to highlight potential future points of intervention, especially with a focus on cardiomyopathies. A review of the function of the Dystrophic Glycoprotein Complex (DGC) in mechanosignaling provides an overview of the various components of DGC and potential mechanopathogenic mechanisms, particularly as they relate to muscular dystrophy.
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影响因子：
16.2
作者：
ADAMS, ME;BUTLER, MH;FROEHNER, SC
通讯作者：
FROEHNER, SC
影响因子：
3.8
作者：
Bhana, Bashir;Iyer, Rohin K.;Radisic, Milica
通讯作者：
Radisic, Milica
影响因子：
14.5
作者：
Alonso-Perez, Jorge;Gonzalez-Quereda, Lidia;Diaz-Manera, Jordi
通讯作者：
Diaz-Manera, Jordi
DOI：
10.36185/2532-1900-062
发表时间：
2021-12
期刊：
Acta myologica : myopathies and cardiomyopathies : official journal of the Mediterranean Society of Myology
影响因子：
--
作者：
Bitetti I;Mautone C;Bertella M;Manna MR;Varone A
通讯作者：
Varone A
影响因子：
2.6
作者：
Boothe, Sean D.;Myers, Jackson D.;Pok, Seokwon;Sun, Junping;Xi, Yutao;Nieto, Raymond M.;Cheng, Jie;Jacot, Jeffrey G.
通讯作者：
Jacot, Jeffrey G.