Department of Molecular Biology and Histocompatibility, "Dr. Manuel Gea González" General Hospital, Mexico City, Mexico.
Faculty of Superior Studies Iztacala, National Autonomous University of Mexico (UNAM), Tlalnepantla, Mexico.
J Membr Biol. 2018 Aug;251(4):535-550. doi: 10.1007/s00232-018-0036-9. Epub 2018 May 19.
Dystrophin is a cytoskeleton-linked membrane protein that binds to a larger multiprotein assembly called the dystrophin-associated glycoprotein complex (DGC). The deficiency of dystrophin or the components of the DGC results in the loss of connection between the cytoskeleton and the extracellular matrix with significant pathophysiological implications in skeletal and cardiac muscle as well as in the nervous system. Although the DGC plays an important role in maintaining membrane stability, it can also be considered as a versatile and flexible molecular complex that contribute to the cellular organization and dynamics of a variety of proteins at specific locations in the plasma membrane. This review deals with the role of the DGC in transmembrane signaling by forming supramolecular assemblies for regulating ion channel localization and activity. These interactions are relevant for cell homeostasis, and its alterations may play a significant role in the etiology and pathogenesis of various disorders affecting muscle and nerve function.
肌营养不良蛋白是一种细胞骨架连接的膜蛋白，与一个称为肌营养不良蛋白相关糖蛋白复合物（DGC）的更大的多蛋白组装体结合。肌营养不良蛋白或 DGC 的成分的缺乏导致细胞骨架与细胞外基质之间的连接丧失，这在骨骼和心肌以及神经系统中具有重要的病理生理意义。尽管 DGC 在维持膜稳定性方面起着重要作用，但它也可以被认为是一个多功能和灵活的分子复合物，有助于在质膜的特定位置处各种蛋白质的细胞组织和动力学。这篇综述涉及 DGC 通过形成超分子组装来调节离子通道定位和活性，从而在跨膜信号转导中发挥作用。这些相互作用与细胞内稳态有关，其改变可能在影响肌肉和神经功能的各种疾病的病因和发病机制中起重要作用。
