Desmin Modulates Muscle Cell Adhesion and Migration.

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.

• Authors: Coralie Hakibilen, Florence Delort, Marie-Thérèse Daher, Pierre Joanne, Eva Cabet, Olivier Cardoso, Fany Bourgois-Rocha, Cuixia Tian, Eloy Rivas, Marcos Madruga, Ana Ferreiro, Alain Lilienbaum, Patrick Vicart, Onnik Agbulut, Sylvie Hénon, Sabrina Batonnet-Pichon
• Journal: Frontiers in cell and developmental biology (Front Cell Dev Biol) Vol. 10 Pg. 783724 ( 2022) ISSN: 2296-634X [Print] Switzerland
• PMID: 35350386 (Publication Type: Journal Article)
• Copyright: Copyright © 2022 Hakibilen, Delort, Daher, Joanne, Cabet, Cardoso, Bourgois-Rocha, Tian, Rivas, Madruga, Ferreiro, Lilienbaum, Vicart, Agbulut, Hénon and Batonnet-Pichon.