%0 Journal Article
%A Hakibilen, Coralie
%A Delort, Florence
%A Daher, Marie-Thérèse
%A Joanne, Pierre
%A Cabet, Eva
%A Cardoso, Olivier
%A Bourgois-Rocha, Fany
%A Tian, Cuixia
%A Rivas, Eloy
%A Madruga, Marcos
%A Ferreiro, Ana
%A Lilienbaum, Alain
%A Vicart, Patrick
%A Agbulut, Onnik
%A Hénon, Sylvie
%A Batonnet-Pichon, Sabrina
%T Desmin Modulates Muscle Cell Adhesion and Migration.
%D 2022
%@ 2296-634X
%U http://hdl.handle.net/10668/20527
%X 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.
%K desmin
%K focal adhesion
%K intermediate filaments
%K migration
%K myopathies
%K vinculin
%~