Intermediate filaments: primary determinants of cell architecture and plasticity

被引:242
作者
Herrmann, Harald [1 ]
Strelkov, Sergei V. [2 ]
Burkhard, Peter [3 ]
Aebi, Ueli [4 ]
机构
[1] DKFZ, German Canc Res Ctr, Grp Funct Architecture Cell, D-69120 Heidelberg, Germany
[2] Katholieke Univ Leuven, Dept Pharmaceut Sci, Louvain, Belgium
[3] Univ Connecticut, Inst Mat Sci, Storrs, CT USA
[4] Univ Basel, Biozentrum, ME Muller Inst Struct Biol, Basel, Switzerland
基金
瑞士国家科学基金会;
关键词
NEMATODE CAENORHABDITIS-ELEGANS; EPIDERMOLYSIS-BULLOSA SIMPLEX; DREIFUSS MUSCULAR-DYSTROPHY; JUVENILE ALEXANDER-DISEASE; DESMIN-RELATED MYOPATHY; MICE LACKING VIMENTIN; IN-VITRO; NUCLEAR-ENVELOPE; COILED-COIL; LAMIN A/C;
D O I
10.1172/JCI38214
中图分类号
R-3 [医学研究方法]; R3 [基础医学];
学科分类号
1001 ;
摘要
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.
引用
收藏
页码:1772 / 1783
页数:12
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