Dystrophin is a protein crucial for maintaining the structural integrity of skeletal muscle. So far, the attention was focused on the role of dystrophin in muscle in view of the devastating progression of weakness and early death that characterises Duchenne muscular dystrophy. However, in the last few years, the role of shorter dystrophin isoforms, including development and adult expression-specific mechanisms, has been a greater focus. Within the cerebral landscape, various cell types, such as glia, oligodendrocytes, Purkinje cells, cerebellar granule cells, and vascular-associated cells express a spectrum of dystrophin isoforms, including Dp427, Dp140, Dp71, and Dp40. The interaction of these isoforms with a multitude of proteins, suggests their involvement in neurotransmission, influencing several circuit functions. This review presents the intricate interactions among dystrophin isoforms and diverse protein complexes across different cell types and brain regions, and the associated clinical complications. We focus on studies investigating protein interactions with dystrophin in the past 30 years, at a biochemical level. In essence, brain's dystrophin landscape is a thrilling exploration of diversity, challenging preconceptions and opening new avenues for understanding the central nervous system physiology. It also holds potential therapeutic implications for neurological complications in which brain dystrophin deficiency is involved. By revealing the molecular complexities related to dystrophin, this review paves the way for future investigations and therapeutic interventions for this central nervous system aspect of Duchenne muscular dystrophy.

中文翻译：

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抗肌萎缩蛋白亚型和相互作用物在大脑中的作用。


抗肌萎缩蛋白是一种对维持骨骼肌结构完整性至关重要的蛋白质。到目前为止，鉴于杜氏肌营养不良症的特征是虚弱和早逝的破坏性进展，人们的注意力集中在抗肌萎缩蛋白在肌肉中的作用上。然而，在过去几年中，较短的抗肌萎缩蛋白亚型的作用，包括发育和成体表达特异性机制，已成为人们更加关注的焦点。在大脑景观中，各种细胞类型，如神经胶质细胞、少突胶质细胞、浦肯野细胞、小脑颗粒细胞和血管相关细胞，表达一系列抗肌萎缩蛋白亚型，包括 Dp427、Dp140、Dp71 和 Dp40。这些亚型与多种蛋白质的相互作用表明它们参与神经传递，影响多种回路功能。本文综述了抗肌萎缩蛋白亚型和不同细胞类型和大脑区域的不同蛋白质复合物之间的复杂相互作用，以及相关的临床并发症。我们专注于在生化水平上调查过去 30 年蛋白质与抗肌萎缩蛋白相互作用的研究。从本质上讲，大脑的抗肌萎缩蛋白景观是对多样性的激动人心的探索，挑战了先入之见，并为理解中枢神经系统生理学开辟了新的途径。它还对涉及脑抗肌萎缩蛋白缺乏症的神经系统并发症具有潜在的治疗意义。通过揭示与抗肌萎缩蛋白相关的分子复杂性，本综述为杜氏肌营养不良症中枢神经系统的未来研究和治疗干预铺平了道路。