Publication:
Desmin Modulates Muscle Cell Adhesion and Migration.

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2022-03-08

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Hakibilen, Coralie
Delort, Florence
Daher, Marie-Thérèse
Joanne, Pierre
Cabet, Eva
Cardoso, Olivier
Bourgois-Rocha, Fany
Tian, Cuixia
Rivas, Eloy
Madruga, Marcos

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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.

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desmin, focal adhesion, intermediate filaments, migration, myopathies, vinculin

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