Laboratory of Synaptogenesis, Nencki Institute of Experimental Biology, Polish Academy of Sciences, Warsaw, Poland.
Ann N Y Acad Sci. 2018 Jan;1412(1):62-72. doi: 10.1111/nyas.13500. Epub 2017 Oct 25.
The proper function of skeletal muscles relies on their ability to process signals derived from motor neurons, transmit stimuli along the muscle fibers, contract, and regenerate efficiently after injury. The dystrophin-glycoprotein complex (DGC; also called the dystrophin-associated protein complex) plays a central role in all of these processes. It acts as a transmembrane platform that anchors the extracellular matrix (ECM) to the intracellular cytoskeleton and makes muscle fibers more resistant to injury. The DGC also contributes to the transmission of contraction-evoked force from the sarcomere to the ECM. The dysfunction of DGC-associated proteins can lead to myopathies, including Duchenne's muscular dystrophy, manifested by progressive muscle damage and impairments in regeneration. The DGC also plays a pivotal role in the organization of neuromuscular junctions (NMJs), where it stabilizes postsynaptic machinery, including receptors for the neurotransmitter acetylcholine (AChRs). Here, we focus on the role of the DGC complex in NMJ and skeletal muscle physiology and discuss the novel components that are associated with the complex.
骨骼肌的正常功能依赖于其处理来自运动神经元信号的能力，沿肌纤维传递刺激，收缩，并在损伤后有效地再生。肌营养不良糖蛋白复合物（DGC；也称为肌营养不良相关蛋白复合物）在所有这些过程中都起着核心作用。它作为一个跨膜平台，将细胞外基质（ECM）固定到细胞内细胞骨架上，并使肌纤维更能抵抗损伤。DGC 还有助于将收缩引起的力从肌节传递到 ECM。DGC 相关蛋白的功能障碍可导致肌病，包括杜兴氏肌营养不良症，其特征是肌肉进行性损伤和再生受损。DGC 还在神经肌肉接头（NMJ）的组织中起关键作用，在 NMJ 中，它稳定突触后机制，包括神经递质乙酰胆碱（AChRs）的受体。在这里，我们专注于 DGC 复合物在 NMJ 和骨骼肌生理学中的作用，并讨论与复合物相关的新成分。
