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发育和疾病中的LINC复合蛋白。

LINC complex proteins in development and disease.

作者信息

Horn Henning F

机构信息

Development and Regenerative Biology, Institute of Medical Biology, Singapore, Singapore.

出版信息

Curr Top Dev Biol. 2014;109:287-321. doi: 10.1016/B978-0-12-397920-9.00004-4.

Abstract

The LINC complex spans the nuclear envelope and plays critical roles in coordinating nuclear and cytoplasmic activities and in organizing nuclear and cytoskeletal features. LINC complexes are assembled from KASH and SUN-domain proteins, which interact in the nuclear envelope and form a physical link between the cytoskeleton and the nuclear interior. A number of diseases have been associated with mutations in genes coding for LINC complex proteins. Mouse models of LINC complex protein have provided valuable insight into LINC complex functions and into how these proteins contribute to the various diseases with which they have been associated.

摘要

LINC复合物跨越核膜,在协调核与细胞质活动以及组织核与细胞骨架特征方面发挥着关键作用。LINC复合物由KASH结构域蛋白和SUN结构域蛋白组装而成,它们在核膜中相互作用,在细胞骨架和核内部之间形成物理连接。许多疾病都与编码LINC复合物蛋白的基因突变有关。LINC复合物蛋白的小鼠模型为深入了解LINC复合物的功能以及这些蛋白如何导致与之相关的各种疾病提供了有价值的见解。

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