AUTHOR=Hakibilen Coralie , Delort Florence , Daher Marie-Thérèse , Joanne Pierre , Cabet Eva , Cardoso Olivier , Bourgois-Rocha Fany , Tian Cuixia , Rivas Eloy , Madruga Marcos , Ferreiro Ana , Lilienbaum Alain , Vicart Patrick , Agbulut Onnik , Hénon Sylvie , Batonnet-Pichon Sabrina TITLE=Desmin Modulates Muscle Cell Adhesion and Migration JOURNAL=Frontiers in Cell and Developmental Biology VOLUME=10 YEAR=2022 URL=https://www.frontiersin.org/journals/cell-and-developmental-biology/articles/10.3389/fcell.2022.783724 DOI=10.3389/fcell.2022.783724 ISSN=2296-634X 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.