期刊
BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH
卷 1867, 期 11, 页码 -出版社
ELSEVIER
DOI: 10.1016/j.bbamcr.2020.118801
关键词
Plakin; Plakin repeat domain; Linker module; Desmoplakin; Arrhythmogenic right ventricular cardiomyopathy; Intermediate filament
资金
- Wellcome Trust [099266/Z/12/Z, 094303/Z/10/Z]
- Campus Alberta Innovates Program [RCP-12-002C]
- Alberta Prion Research Institute/Alberta Innovates Bio Solutions [201600018]
- NSERC [RGPIN-2018-04994]
- Wellcome Trust [094303/Z/10/Z] Funding Source: Wellcome Trust
The plakin family of cytolinkers interacts with intermediate filaments (IFs) through plakin repeat domain (PRD) and linker modules. Recent structure/function studies have established the molecular basis of envoplakin-PRD and periplakin-linker interactions with vimentin. Both plakin modules share a broad basic groove which recognizes acidic rod elements on IFs, a mechanism that is applicable to other plakin family members. This review postulates a universal IF engagement mechanism that illuminates the specific effects of pathogenic mutations associated with diseases including arrhythmogenic right ventricular cardiomyopathy, and reveals how diverse plakin proteins offer tailored IF tethering to ensure stable, dynamic and regulated cellular structures.
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