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- W765285249 abstract "Desminopathies are neuromuscular genetic diseases caused by mutations in the desmin gene. They are characterized by the presence in muscles of aggregates containing desmin and by degenerative changes of the contractile apparatus. Although desminopathies have been largely studied at clinical level, the different steps that lead from a desmin gene mutation to progressive muscle weakness are stiil unclear. We investigated this problem in early stages of disease pathology and within an isogenic background, by using C2C12 myoblasts electroporated with the E413K mutant desmin. We first show that the expression of this mutant induces a large desmin network disorganization associated with important aggregate formation. We also compared the mechanical properties of wild-type C2C12 cells, cells over-expressing desmin-WT-GFP and cells expressing mutated desmin E413K-GFP. We show that the three cell types share similar visco-elastic moduli of the cortex, whereas expression of WT-desmin -but not of mutated desmin — increases the overall rigidity of cells. We finally investigated the impact of mutated desmin on the contractility of myoblasts in two different geometries, with a custom-made single cell technique, and with Traction Force Microscopy. We show that E413K-mutation significantly decreases cell contraction abilities. We thus demonstrate for the first time that the impaired contractile strength of muscles observed in desminopathies is already present at very early stage, in isolated myoblasts and at very short time of mutated desmin expression. Finally we have begun to investigate the effect of E413K mutated desmin expression on engineered microtissues made of C2C12 myoblasts." @default.
- W765285249 created "2016-06-24" @default.
- W765285249 creator A5074833641 @default.
- W765285249 date "2014-01-01" @default.
- W765285249 modified "2023-09-27" @default.
- W765285249 title "Implication de la desmine dans les propriétés mécaniques des cellules musculaires squelettiques dans le contexte des desminopathies" @default.
- W765285249 hasPublicationYear "2014" @default.
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