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Nuclear Lamins: Thin Filaments with Major Functions
Trends in Cell Biology ( IF 19.0 ) Pub Date : 2017-09-08 , DOI: 10.1016/j.tcb.2017.08.004
Rebecca de Leeuw , Yosef Gruenbaum , Ohad Medalia

The nuclear lamina is a nuclear peripheral meshwork that is mainly composed of nuclear lamins, although a small fraction of lamins also localizes throughout the nucleoplasm. Lamins are classified as type V intermediate filament (IF) proteins. Mutations in lamin genes cause at least 15 distinct human diseases, collectively termed laminopathies, including muscle, metabolic, and neuronal diseases, and can cause accelerated aging. Most of these mutations are in the LMNA gene encoding A-type lamins. A growing number of nuclear proteins are known to bind lamins and are implicated in both nuclear and cytoskeletal organization, mechanical stability, chromatin organization, signaling, gene regulation, genome stability, and cell differentiation. Recent studies reveal the organization of the lamin filament meshwork in somatic cells where they assemble as tetramers in cross-section of the filaments.



中文翻译:

核纤层:具有主要功能的细丝

核薄层是主要由核薄层组成的核外围网,尽管一小部分薄层也位于整个核质中。核纤层蛋白被归类为V型中间丝(IF)蛋白。核纤层蛋白基因的突变会导致至少15种不同的人类疾病(统称为肌肉疾病),包括肌肉,代谢和神经元疾病,并可能导致加速衰老。这些突变大多数在LMNA中编码A型核纤层蛋白的基因。已知越来越多的核蛋白与纤溶蛋白结合,并参与核和细胞骨架组织,机械稳定性,染色质组织,信号传导,基因调控,基因组稳定性和细胞分化。最近的研究揭示了在体细胞中层状细丝网状组织,它们在细丝的横截面中以四聚体的形式组装。

更新日期:2017-09-08
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