Desmin Modulates Muscle Cell Adhesion and Migration

Coralie Hakibilen (1) , Florence Delort (1) , Marie-Thérèse Daher (2) , Pierre Joanne (3) , Eva Cabet (1) , Olivier Cardoso (4) , Fany Bourgois-Rocha (1) , Cuixia Tian , Eloy Rivas (5) , Marcos Madruga , Ana Ferreiro , Alain Lilienbaum (1) , Patrick Vicart (1) , Onnik Agbulut (3) , Sylvie Hénon , Sabrina Batonnet-Pichon (1)

1 BFA (UMR_8251 / U1133) - Unité de Biologie Fonctionnelle et Adaptative
2 Centre de recherche en Myologie – U974 SU-INSERM
3 B2A-IBPS - Adaptation Biologique et Vieillissement = Biological Adaptation and Ageing
4 OFB - Office français de la biodiversité
5 Hospital Universitario Virgen del Rocío [Sevilla]

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.

Domaines: Biophysique [physics.bio-ph]

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-03876281⟩