Lowery Jason, Kuczmarski Edward R, Herrmann Harald, Goldman Robert D
From the Department of Cell and Molecular Biology, Feinberg School of Medicine, Northwestern University, Chicago, Illinois 60611 and.
the Division of Molecular Genetics (B060), German Cancer Research Center (DKFZ), D-69120 Heidelberg, Germany.
J Biol Chem. 2015 Jul 10;290(28):17145-53. doi: 10.1074/jbc.R115.640359. Epub 2015 May 8.
Intermediate filaments (IFs) are composed of one or more members of a large family of cytoskeletal proteins, whose expression is cell- and tissue type-specific. Their importance in regulating the physiological properties of cells is becoming widely recognized in functions ranging from cell motility to signal transduction. IF proteins assemble into nanoscale biopolymers with unique strain-hardening properties that are related to their roles in regulating the mechanical integrity of cells. Furthermore, mutations in the genes encoding IF proteins cause a wide range of human diseases. Due to the number of different types of IF proteins, we have limited this short review to cover structure and function topics mainly related to the simpler homopolymeric IF networks composed of vimentin, and specifically for diseases, the related muscle-specific desmin IF networks.
中间丝(IFs)由一大类细胞骨架蛋白中的一种或多种成员组成,其表达具有细胞和组织类型特异性。它们在调节细胞生理特性方面的重要性在从细胞运动到信号转导等一系列功能中得到了广泛认可。IF蛋白组装成具有独特应变硬化特性的纳米级生物聚合物,这与其在调节细胞机械完整性中的作用有关。此外,编码IF蛋白的基因突变会导致多种人类疾病。由于IF蛋白类型众多,我们将本简短综述限制在主要涉及由波形蛋白组成的更简单的同聚物IF网络的结构和功能主题上,特别是对于疾病,还涉及相关的肌肉特异性结蛋白IF网络。