Mechanotransduction translates forces into biological responses and regulates cell functionalities. It is implicated in several diseases, including laminopathies which are pathologies associated with mutations in lamins and lamin-associated proteins. These pathologies affect muscle, adipose, bone, nerve, and skin cells and range from muscular dystrophies to accelerated aging. Although the exact mechanisms governing laminopathies and gene expression are still not clear, a strong correlation has been found between cell functionality and nuclear behavior. New theories base on the direct effect of external force on the genome, which is indeed sensitive to the force transduced by the nuclear lamina. Nuclear lamina performs two essential functions in mechanotransduction pathway modulating the nuclear stiffness and governing the chromatin remodeling. Indeed, A-type lamin mutation and deregulation has been found to affect the nuclear response, altering several downstream cellular processes such as mitosis, chromatin organization, DNA replication-transcription, and nuclear structural integrity. In this review, we summarize the recent findings on the molecular composition and architecture of the nuclear lamina, its role in healthy cells and disease regulation. We focus on A-type lamins since this protein family is the most involved in mechanotransduction and laminopathies.

中文翻译：

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椎板狭窄的椎板A / C机械转导。

机械转导将力转化为生物反应并调节细胞功能。它与多种疾病有关，包括与椎板蛋白和层粘连蛋白相关的蛋白质突变相关的病理学。这些病理影响肌肉，脂肪，骨骼，神经和皮肤细胞，范围从肌肉营养不良到加速衰老。尽管尚不清楚控制椎间盘突出症和基因表达的确切机制，但已发现细胞功能与核行为之间有很强的相关性。新的理论基于外力对基因组的直接影响，而外力对基因组核膜层转导的力确实敏感。核层在机械转导途径中调节核劲度并控制染色质重塑中执行两个基本功能。实际上，已发现A型核纤层蛋白突变和失调会影响核反应，从而改变了一些下游细胞过程，例如有丝分裂，染色质组织，DNA复制转录和核结构完整性。在这篇综述中，我们总结了有关核层的分子组成和结构，其在健康细胞和疾病调控中的作用的最新发现。由于该蛋白家族与机械转导和laminopathies相关程度最高，因此我们重点研究A型lamin。我们总结了有关核层的分子组成和结构，其在健康细胞和疾病调控中的作用的最新发现。由于该蛋白家族与机械转导和laminopathies相关程度最高，因此我们重点研究A型lamin。我们总结了有关核层的分子组成和结构，其在健康细胞和疾病调控中的作用的最新发现。由于该蛋白家族与机械转导和laminopathies相关程度最高，因此我们重点研究A型lamin。