Muscle fibres are very specialised cells with a complex structure that requires a high level of organisation of the constituent proteins. For muscle contraction to function properly, there is a need for not only sarcomeres, the contractile structures of the muscle fibre, but also costameres. These are supramolecular structures associated with the sarcolemma that allow muscle adhesion to the extracellular matrix. They are composed of protein complexes that interact and whose functions include maintaining cell structure and signal transduction mediated by their constituent proteins. It is important to improve our understanding of these structures, as mutations in various genes that code for costamere proteins cause many types of muscular dystrophy. In this review, we provide a description of costameres detailing each of their constituent proteins, such as dystrophin, dystrobrevin, syntrophin, sarcoglycans, dystroglycans, vinculin, talin, integrins, desmin, plectin, etc. We describe as well the diseases associated with deficiency thereof, providing a general overview of their importance.

中文翻译：

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Costamere 蛋白及其在肌病过程中的作用

肌肉纤维是非常特殊的细胞，具有复杂的结构，需要高水平的组成蛋白质组织。为了使肌肉收缩正常发挥作用，不仅需要肌节，即肌纤维的收缩结构，还需要肋节。这些是与肌膜相关的超分子结构，允许肌肉粘附到细胞外基质。它们由相互作用的蛋白质复合物组成，其功能包括维持细胞结构和由其组成蛋白质介导的信号转导。提高我们对这些结构的理解很重要，因为编码肋粒蛋白的各种基因的突变会导致多种类型的肌营养不良症。在这篇评论中，我们提供了对肋骨的描述，详细说明了它们的每一种组成蛋白质，