Nesprins: from the nuclear envelope and beyond.

Nesprins: from the nuclear envelope and beyond.
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DOI:
10.1017/erm.2013.6
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发表时间:
2013-07-05
影响因子:
6.2
通讯作者:
Shanahan CM
Shanahan CM
中科院分区:
医学2区
文献类型:
--
作者:
Rajgor D;Shanahan CM

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核膜血影蛋白重复序列蛋白(Nesprins)是一类新的核蛋白和细胞骨架蛋白,作为细胞内支架和连接蛋白的作用正在迅速扩大。nesprins最初被描述为定位于核膜(NE)并建立核-细胞骨架连接的蛋白质,现在已发现其包含定位于多个亚细胞区室的多种组织特异性同种型。在这里,我们描述了如何nesprins是必要的,在维持细胞的结构,作为必要的支架和连接器在NE和其他亚细胞域。更重要的是,我们推测nesprin突变如何破坏组织特异性nesprin支架,并解释许多nesprin相关疾病(包括核纤层蛋白病)的组织特异性。
Nuclear envelope spectrin-repeat proteins (Nesprins), are a novel family of nuclear and cytoskeletal proteins with rapidly expanding roles as intracellular scaffolds and linkers. Originally described as proteins that localise to the nuclear envelope (NE) and establish nuclear-cytoskeletal connections, nesprins have now been found to comprise a diverse spectrum of tissue specific isoforms that localise to multiple sub-cellular compartments. Here, we describe how nesprins are necessary in maintaining cellular architecture by acting as essential scaffolds and linkers at both the NE and other sub-cellular domains. More importantly, we speculate how nesprin mutations may disrupt tissue specific nesprin scaffolds and explain the tissue specific nature of many nesprin-associated diseases, including laminopathies.
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发表时间: 2010-01-29
期刊: The Journal of biological chemistry
影响因子: --
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