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Journal Articles Frontiers in Cell and Developmental Biology Year : 2022

Desmin Modulates Muscle Cell Adhesion and Migration

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Coralie Hakibilen
  • Function : Author
Florence Delort
  • Function : Author
Marie-Thérèse Daher
Pierre Joanne
Eva Cabet
  • Function : Author
Olivier Cardoso
  • Function : Author
Fany Bourgois-Rocha
  • Function : Author
Cuixia Tian
  • Function : Author
Eloy Rivas
  • Function : Author
Marcos Madruga
  • Function : Author
Ana Ferreiro
  • Function : Author
Alain Lilienbaum
  • Function : Author
Patrick Vicart
  • Function : Author
Onnik Agbulut
Sabrina Batonnet-Pichon
  • Function : Author

Abstract

Cellular adhesion and migration are key functions that are disrupted in numerous diseases. We report that desmin, a type-III muscle-specific intermediate filament, is a novel cell adhesion regulator. Expression of p.R406W mutant desmin, identified in patients with desmin-related myopathy, modified focal adhesion area and expression of adhesion-signaling genes in myogenic C2C12 cells. Satellite cells extracted from desmin-knock-out (DesKO) and desmin-knock-in-p.R405W (DesKI-R405W) mice were less adhesive and migrated faster than those from wild-type mice. Moreover, we observed mislocalized and aggregated vinculin, a key component of cell adhesion, in DesKO and DesKI-R405W muscles. Vinculin expression was also increased in desmin-related myopathy patient muscles. Together, our results establish a novel role for desmin in cell-matrix adhesion, an essential process for strength transmission, satellite cell migration and muscle regeneration. Our study links the patho-physiological mechanisms of desminopathies to adhesion/migration defects, and may lead to new cellular targets for novel therapeutic approaches.

Dates and versions

hal-03669613 , version 1 (16-05-2022)

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Coralie Hakibilen, Florence Delort, Marie-Thérèse Daher, Pierre Joanne, Eva Cabet, et al.. Desmin Modulates Muscle Cell Adhesion and Migration. Frontiers in Cell and Developmental Biology, 2022, 10, ⟨10.3389/fcell.2022.783724⟩. ⟨hal-03669613⟩
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