Herrmann Harald, Strelkov Sergei V, Burkhard Peter, Aebi Ueli
Group Functional Architecture of the Cell, German Cancer Research Center, Heidelberg, Germany.
J Clin Invest. 2009 Jul;119(7):1772-83. doi: 10.1172/JCI38214. Epub 2009 Jul 1.
Intermediate filaments (IFs) are major constituents of the cytoskeleton and nuclear boundary in animal cells. They are of prime importance for the functional organization of structural elements. Depending on the cell type, morphologically similar but biochemically distinct proteins form highly viscoelastic filament networks with multiple nanomechanical functions. Besides their primary role in cell plasticity and their established function as cellular stress absorbers, recently discovered gene defects have elucidated that structural alterations of IFs can affect their involvement both in signaling and in controlling gene regulatory networks. Here, we highlight the basic structural and functional properties of IFs and derive a concept of how mutations may affect cellular architecture and thereby tissue construction and physiology.
中间丝（IFs）是动物细胞细胞骨架和核边界的主要组成部分。它们对于结构元件的功能组织至关重要。根据细胞类型的不同，形态相似但生化性质不同的蛋白质形成具有多种纳米力学功能的高粘弹性丝状网络。除了在细胞可塑性方面的主要作用以及作为细胞应力吸收器的既定功能外，最近发现的基因缺陷表明，中间丝的结构改变会影响它们在信号传导和控制基因调控网络中的参与。在这里，我们强调中间丝的基本结构和功能特性，并得出一个关于突变如何影响细胞结构从而影响组织结构和生理学的概念。
