Hakibilen, CoralieDelort, FlorenceDaher, Marie-ThérèseJoanne, PierreCabet, EvaCardoso, OlivierBourgois-Rocha, FanyTian, CuixiaRivas, EloyMadruga, MarcosFerreiro, AnaLilienbaum, AlainVicart, PatrickAgbulut, OnnikHénon, SylvieBatonnet-Pichon, Sabrina2023-05-032023-05-032022-03-082296-634Xhttp://hdl.handle.net/10668/20527Cellular 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.enAttribution 4.0 Internationalhttp://creativecommons.org/licenses/by/4.0/desminfocal adhesionintermediate filamentsmigrationmyopathiesvinculinresearch article35350386open access10.3389/fcell.2022.783724PMC8957967https://www.frontiersin.org/articles/10.3389/fcell.2022.783724/pdfhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC8957967/pdf