Dystrophin supports muscle fiber strength, and the absence of dystrophin reduces muscle stiffness, increases sarcolemmal deformability, and compromises the mechanical stability of costameres and their connections to nearby myofibrils.

Dystrophin is a 427kDa cytoskeletal protein that localizes to the cytoplasmic face of the sarcolemma and is enriched at costameres in muscle fibers . Dystrophin protein has four main functional domains; an actin-binding amino-terminal domain (ABD1), a central rod domain, a cysteine-rich domain and a carboxyl-terminus .

Sep 27, 2022 · 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).

DMD基因编码dystrophin蛋白（抗肌萎缩蛋白），dystrophin主要分布于骨骼肌和心肌细胞，通过其N端和C端分别与细胞骨架和细胞膜，起支架作用，可以保护肌细胞膜在肌肉收缩时不受损伤。

这是由于该疾病的致病基因十分明确，有且只有一个，就是dystrophin，中文名既可以是肌营养不良蛋白，又可以叫做抗肌萎缩蛋白。 该基因在保证人类正常肌肉结构和功能中是不可或缺的。

如何选择合适的micro-dystrophin是基因治疗的一个关键问题。研究团队所解析的结构显示，在dystrophin上占全长不足1/10长度的CR区域（cysteine-rich domain，图3橙色区域）是dystrophin参与DGC组装最核心区域。该结果为设计更加合理有效的micro-dystrophin提供了重要参考。

Dystrophin is a cytosolic protein associated to a glycoprotein complex, the dystrophin–glycoprotein complex (DGC), composed by the intracellular α and β-syntrophin, α-dystrobrevin and neuronal nitric oxide synthase (nNOS), the transmembrane β-dystroglycan, α-, β-, γ- and δ-sarcoglycan and sarcospan, and the extracellular α ...

The largest transcripts encode a four-domain protein with a predicted molecular weight of 427,000, named dystrophin. Dystrophin is the predominant DMD transcript expressed in striated muscle and DMD gene mutations, deletions or duplications most frequently result in a loss of dystrophin expression in muscle of patients afflicted with DMD.

The dystrophin gene (DMD) encodes dystrophin, a large cytoskeletal protein expressed predominantly in skeletal, heart, and smooth muscle. The dystrophin protein interacts with both cytoskeletal actin and the dystrophin-associated glycoprotein complex, which provide a strong mechanical link from the intracellular cytoskeleton to the ECM.

肌营养不良蛋白糖蛋白复合物（dystrophin glycoprotein complex，DGC）通过连接细胞内细胞骨架和周围的细胞外基质，在维持细胞膜的稳定性和完整性方面起着至关重要的作用。肌营养不良蛋白及其相关蛋白的功能障碍导…