ABSTRACT The nuclear envelope compartmentalizes chromatin in eukaryotic cells. The main nuclear envelope components are lamins that associate with a panoply of factors, including the LEM domain proteins. The nuclear envelope of mammalian cells opens up during cell division. It is reassembled and associated with chromatin at the end of mitosis when telomeres tether to the nuclear periphery. Lamins, LEM domain proteins, and DNA binding factors, as BAF, contribute to the reorganization of chromatin. In this context, an emerging role is that of the ESCRT complex, a machinery operating in multiple membrane assembly pathways, including nuclear envelope reformation. Research in this area is unraveling how, mechanistically, ESCRTs link to nuclear envelope associated factors as LEM domain proteins. Importantly, ESCRTs work also during interphase for repairing nuclear envelope ruptures. Altogether the advances in this field are giving new clues for the interpretation of diseases implicating nuclear envelope fragility, as laminopathies and cancer. Abbreviations na, not analyzed; ko, knockout; kd, knockdown; NE, nuclear envelope; LEM, LAP2-emerin-MAN1 (LEM)-domain containing proteins; LINC, linker of nucleoskeleton and cytoskeleton complexes; Cyt, cytoplasm; Chr, chromatin; MB, midbody; End, endosomes; Tel, telomeres; INM, inner nuclear membrane; NP, nucleoplasm; NPC, Nuclear Pore Complex; ER, Endoplasmic Reticulum; SPB, spindle pole body.

中文翻译：

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核膜与染色质在生理学和病理学中的相互作用

摘要 核膜将真核细胞中的染色质区分开来。主要的核被膜成分是与一系列因素相关的核纤层蛋白，包括 LEM 结构域蛋白。哺乳动物细胞的核膜在细胞分裂过程中打开。当端粒与核外围相连时，它会在有丝分裂末期重新组装并与染色质相关联。层蛋白、LEM 结构域蛋白和 DNA 结合因子，作为 BAF，有助于染色质的重组。在这种情况下，一个新兴的角色是 ESCRT 复合体，这是一种在多个膜组装途径中运行的机器，包括核膜重整。该领域的研究正在解开 ESCRT 如何与作为 LEM 结构域蛋白的核膜相关因子相关联的机制。重要的，ESCRT 也在相间期工作以修复核包膜破裂。总之，该领域的进展为解释与核膜脆性有关的疾病（如椎板病和癌症）提供了新的线索。缩写 na，未分析；ko，淘汰赛；kd，击倒；NE，核膜；LEM，含有 LAP2-emerin-MAN1 (LEM) 结构域的蛋白质；LINC，核骨架和细胞骨架复合物的接头；Cyt，细胞质；Chr，染色质；MB，中体；末端，内体；电话，端粒；INM，内核膜；NP，核质；NPC，核孔复合体；ER，内质网；SPB，主轴杆体。未分析；ko，淘汰赛；kd，击倒；NE，核膜；LEM，含有 LAP2-emerin-MAN1 (LEM) 结构域的蛋白质；LINC，核骨架和细胞骨架复合物的接头；Cyt，细胞质；Chr，染色质；MB，中体；末端，内体；电话，端粒；INM，内核膜；NP，核质；NPC，核孔复合体；ER，内质网；SPB，主轴杆体。未分析；ko，淘汰赛；kd，击倒；NE，核膜；LEM，含有 LAP2-emerin-MAN1 (LEM) 结构域的蛋白质；LINC，核骨架和细胞骨架复合物的接头；Cyt，细胞质；Chr，染色质；MB，中体；末端，内体；电话，端粒；INM，内核膜；NP，核质；NPC，核孔复合体；ER，内质网；SPB，主轴杆体。