期刊
MOLECULAR BIOLOGY OF THE CELL
卷 30, 期 5, 页码 579-590出版社
AMER SOC CELL BIOLOGY
DOI: 10.1091/mbc.E18-11-0718
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资金
- Institut National de la Sante et de la Recherche Medicale (INSERM)
- Association Institut de Myologie (AIM)
- Agence Nationale de la Recherche grant [ANR-14-CE12-0009]
- Sorbonne Universite (SU)
- [ANR-14-CE12-0001-01]
- Agence Nationale de la Recherche (ANR) [ANR-14-CE12-0009] Funding Source: Agence Nationale de la Recherche (ANR)
Clathrin plaques are stable features of the plasma membrane observed in several cell types. They are abundant in muscle, where they localize at costameres that link the contractile apparatus to the sarcolemma and connect the sarcolemma to the basal lamina. Here, we show that clathrin plaques and surrounding branched actin filaments form microdomains that anchor a three-dimensional desmin intermediate filament (IF) web. Depletion of clathrin plaque and branched actin components causes accumulation of desmin tangles in the cytoplasm. We show that dynamin 2, whose mutations cause centronuclear myopathy (CNM), regulates both clathrin plaques and surrounding branched actin filaments, while CNM-causing mutations lead to desmin disorganization in a CNM mouse model and patient biopsies. Our results suggest a novel paradigm in cell biology, wherein clathrin plaques act as platforms capable of recruiting branched cortical actin, which in turn anchors IFs, both essential for striated muscle formation and function.
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