Intermediate Filaments: Structure and Assembly
Intermediate Filaments: Structure and Assembly
复制标题
DOI：
10.1101/cshperspect.a018242
复制
发表时间：
2016-11-01
影响因子：
7.2
通讯作者：
Aebi, Ueli
中科院分区：
文献类型：
--
作者：
Herrmann, Harald;Aebi, Ueli
中间丝（IF）超基因家族的蛋白质是普遍存在的结构组分，其以细胞类型特异性的方式包含动物组织中的细胞骨架。所有IF蛋白显示出明显组织化的、延伸的α-螺旋构象，易于形成双链卷曲螺旋，这是这些高度柔性的、抗应力的细胞骨架丝的基本构建块。IF蛋白质是高度带电的，因此代表具有多种功能的通用聚两性电解质。以波形蛋白、角蛋白和核纤层蛋白为例，综述了它们的分子结构参数。这些，反过来，文件的IF蛋白形成不同的，高度多样化的超分子组件和生物材料的能力，发现，例如，在内核膜，整个细胞质，并在高度复杂的细胞外附属物，如头发和指甲，脊椎动物生物体。最终，我们的目标是为更合理地理解IF基因的错义突变对细胞功能的直接影响以及它们对由它们引起的众多IF疾病的发展的深远影响奠定基础。
Proteins of the intermediate filament (IF) supergene family are ubiquitous structural components that comprise, in a cell type-specific manner, the cytoskeleton proper in animal tissues. All IF proteins show a distinctly organized, extended a-helical conformation prone to form two-stranded coiled coils, which are the basic building blocks of these highly flexible, stressresistant cytoskeletal filaments. IF proteins are highly charged, thus representing versatile polyampholytes with multiple functions. Taking vimentin, keratins, and the nuclear lamins as our prime examples, we present an overview of their molecular and structural parameters. These, in turn, document the ability of IF proteins to form distinct, highly diverse supramolecular assemblies and biomaterials found, for example, at the inner nuclear membrane, throughout the cytoplasm, and in highly complex extracellular appendages, such as hair and nails, of vertebrate organisms. Ultimately, our aim is to set the stage for a more rational understanding of the immediate effects that missense mutations in IF genes have on cellular functions and for their far-reaching impact on the development of the numerous IF diseases caused by them.