Desmin Modulates Muscle Cell Adhesion and Migration.
desmin
focal adhesion
intermediate filaments
migration
myopathies
vinculin
Journal
Frontiers in cell and developmental biology
ISSN: 2296-634X
Titre abrégé: Front Cell Dev Biol
Pays: Switzerland
ID NLM: 101630250
Informations de publication
Date de publication:
2022
2022
Historique:
received:
26
09
2021
accepted:
09
02
2022
entrez:
30
3
2022
pubmed:
31
3
2022
medline:
31
3
2022
Statut:
epublish
Résumé
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.
Identifiants
pubmed: 35350386
doi: 10.3389/fcell.2022.783724
pii: 783724
pmc: PMC8957967
doi:
Types de publication
Journal Article
Langues
eng
Pagination
783724Informations de copyright
Copyright © 2022 Hakibilen, Delort, Daher, Joanne, Cabet, Cardoso, Bourgois-Rocha, Tian, Rivas, Madruga, Ferreiro, Lilienbaum, Vicart, Agbulut, Hénon and Batonnet-Pichon.
Déclaration de conflit d'intérêts
The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.
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