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Nucleoskeleton dynamics and functions in health and disease

机译：健康和疾病中的核骨骼动力学和功能

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Abstract: It is a common misconception to view the "cyto"-skeleton as just the filament systems in the "cyto"-plasm. In fact, the cytoskeleton extends into the nucleus where the complex network connects to chromatin, and it also connects through the plasma membrane to the cytoskeleton of adjacent cells and to the "exo"-skeleton of the extracellular matrix. This review will focus principally on the subcomplex of the cytoskeleton associated with the nucleus, often referred to as the nucleoskeleton, but in the context of its extensive interconnectivity with the rest of the nucleus and with cytoplasmic filament systems all the way to the exoskeleton. The nucleoskeleton, made principally of type-V intermediate filament lamins, connects across the double membrane system of the nuclear envelope to likely all three primary cytoplasmic filament systems. It provides structural stability to the nucleus, and also incredible flexibility. In both its core structural aspect and through specificity gained by tissue-specific partner proteins, it contributes to genome organization and regulation as well as to signal transduction, both through chemical signaling cascades and likely through mechanotransduction. Defects in the nucleoskeleton have far-ranging effects due to its interactions with cytoplasmic filament systems, from mispositioning of nuclei to disruption of cell polarity and both decreased and increased cell migration depending on the defect. Accordingly, it is not surprising that many nucleoskeletal components are linked to a wide range of human diseases from specific types of cancer to muscular dystrophies, neuropathies, dermopathies, and premature aging syndromes.
机译：摘要：将“细胞”骨架视为“细胞”质中的细丝系统是一个普遍的误解。实际上，细胞骨架延伸到核中，复杂网络连接到染色质，并且还通过质膜连接到相邻细胞的细胞骨架，并连接到细胞外基质的“ exo”骨架。这篇综述主要集中在与核相关的细胞骨架的亚复合体上，通常被称为核骨架，但是在其与核的其余部分以及一直到外骨骼的细胞质细丝系统的广泛互连的背景下。核骨架主要由V型中间丝纤层制成，跨整个核膜的双膜系统连接到可能的所有三个主要细胞质丝系统。它为核提供了结构稳定性，并提供了难以置信的灵活性。在其核心结构方面和通过组织特异性伴侣蛋白获得的特异性，它都通过化学信号级联和可能通过机械转导，促进了基因组的组织和调控以及信号转导。由于其与胞质细丝系统的相互作用，核骨架中的缺陷具有广泛的影响，从核的位置错误到细胞极性的破坏，以及取决于缺陷的细胞迁移的减少和增加。因此，不足为奇的是，许多核骨架成分与多种人类疾病相关，从特定类型的癌症到肌肉营养不良，神经病，皮肤病和早衰综合症。

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