Department of Animal Developmental Biology, University of Wroclaw, 21 Sienkiewicza Street, 50-335, Wroclaw, Poland.
Cell Mol Life Sci. 2013 Aug;70(15):2713-41. doi: 10.1007/s00018-012-1190-3. Epub 2012 Nov 10.
The aim of this review article is to evaluate the current knowledge on associations between muscle formation and regeneration and components of the nuclear lamina. Lamins and their partners have become particularly intriguing objects of scientific interest since it has been observed that mutations in genes coding for these proteins lead to a wide range of diseases called laminopathies. For over the last 10 years, various laboratories worldwide have tried to explain the pathogenesis of these rare disorders. Analyses of the distinct aspects of laminopathies resulted in formulation of different hypotheses regarding the mechanisms of the development of these diseases. In the light of recent discoveries, A-type lamins--the main building blocks of the nuclear lamina--together with other key elements, such as emerin, LAP2α and nesprins, seem to be of great importance in the modulation of various signaling pathways responsible for cellular differentiation and proliferation.
这篇综述文章的目的是评估目前关于肌肉形成和再生与核层成分之间关联的知识。自从观察到编码这些蛋白质的基因突变会导致称为层粘连蛋白病的多种疾病以来,层粘连蛋白及其伴侣已成为科学研究特别有趣的对象。在过去的 10 年中,世界各地的各个实验室都试图解释这些罕见疾病的发病机制。对层粘连蛋白病不同方面的分析导致了对这些疾病发展机制的不同假设的形成。根据最近的发现,A 型核纤层蛋白——核层的主要组成部分——与其他关键元素(如 emerin、LAP2α 和 nesprins)一起,似乎在调节各种信号通路方面非常重要,这些信号通路负责细胞分化和增殖。