RT Journal Article
T1 Desmin Modulates Muscle Cell Adhesion and Migration.
A1 Hakibilen, Coralie
A1 Delort, Florence
A1 Daher, Marie-Thérèse
A1 Joanne, Pierre
A1 Cabet, Eva
A1 Cardoso, Olivier
A1 Bourgois-Rocha, Fany
A1 Tian, Cuixia
A1 Rivas, Eloy
A1 Madruga, Marcos
A1 Ferreiro, Ana
A1 Lilienbaum, Alain
A1 Vicart, Patrick
A1 Agbulut, Onnik
A1 Hénon, Sylvie
A1 Batonnet-Pichon, Sabrina
K1 desmin
K1 focal adhesion
K1 intermediate filaments
K1 migration
K1 myopathies
K1 vinculin
AB 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.
SN 2296-634X
YR 2022
FD 2022-03-08
LK http://hdl.handle.net/10668/20527
UL http://hdl.handle.net/10668/20527
LA en
DS RISalud
RD Apr 9, 2025