Dystrophin-associated proteins in muscular dystrophy.
Dystrophin-associated proteins in muscular dystrophy.
复制标题
肌营养不良症中的肌营养不良蛋白相关蛋白。
作者：
E. Ozawa;Mikiharu Yoshida;A. Suzuki;Y. Mizuno;Y. Hagiwara;S. Noguchi
肌营养不良蛋白相关蛋白（Dystrophin-associated proteins，DAP）分为几类，即肌营养不良蛋白聚糖复合物（dystroglycan complex）、肌聚糖复合物（sarcoglycan complex）、突触营养蛋白复合物（syntrophin complex）等。肌膜下肌动蛋白丝通过肌营养不良蛋白和肌营养不良蛋白聚糖复合物与基底膜层粘连蛋白连接。该系统可以起到保护肌纤维免受机械损伤的作用。此外，肌聚糖复合物与系统相关。保护系统或肌聚糖复合物或两者的组分缺陷在各种肌营养不良症中特征性地发现。对突触营养蛋白复合体的作用了解甚少。在这篇综述中，层粘连蛋白，DAP和肌营养不良蛋白在每一个营养不良的可能作用进行了解释。
Dystrophin-associated proteins (DAPs) are classified into a few groups, namely, those comprising of dystroglycan complex, sarcoglycan complex, syntrophin complex and others. Subsarcolemmal actin filaments are connected to laminin in the basement membrane through dystrophin and the dystroglycan complex. This system may function to protect muscle fibers from mechanical damage. Furthermore, the sarcoglycan complex is associated with the system. Defects in the components of the protection system or the sarcoglycan complex or both are characteristically found in various muscular dystrophies. The roles of the syntrophin complex are meagerly understood. In this review, the possible roles of laminin, DAPs and dystrophin in each dystrophy are explained.