Desmin Mutation in the C-Terminal Domain Impairs Traction Force Generation in Myoblasts

Biophysical Journal
Elisabeth E CharrierSylvie Hénon

Abstract

The cytoskeleton plays a key role in the ability of cells to both resist mechanical stress and generate force, but the precise involvement of intermediate filaments in these processes remains unclear. We focus here on desmin, a type III intermediate filament, which is specifically expressed in muscle cells and serves as a skeletal muscle differentiation marker. By using several complementary experimental techniques, we have investigated the impact of overexpressing desmin and expressing a mutant desmin on the passive and active mechanical properties of C2C12 myoblasts. We first show that the overexpression of wild-type-desmin increases the overall rigidity of the cells, whereas the expression of a mutated E413K desmin does not. This mutation in the desmin gene is one of those leading to desminopathies, a subgroup of myopathies associated with progressive muscular weakness that are characterized by the presence of desmin aggregates and a disorganization of sarcomeres. We show that the expression of this mutant desmin in C2C12 myoblasts induces desmin network disorganization, desmin aggregate formation, and a small decrease in the number and total length of stress fibers. We finally demonstrate that expression of the E413K mutant...Continue Reading

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Citations

Jun 22, 2018·Biophysics Reviews·Andreas BrodehlHendrik Milting
Oct 6, 2017·Cell Biology International·Wolfgang H Goldmann
Oct 19, 2019·Cytoskeleton·Yoshiya MiyasakaKuniyuki Hatori
Feb 2, 2018·Biology of the Cell·Elisabeth E CharrierSylvie Hénon
Jan 18, 2020·Stem Cells and Development·Yaqi WangDapeng Ren
Apr 8, 2020·Proceedings of the National Academy of Sciences of the United States of America·Jing W HughesDavid W Piston
May 26, 2021·Current Biology : CB·Gaëlle Dutour-Provenzano, Sandrine Etienne-Manneville
Dec 2, 2021·Neuropathology and Applied Neurobiology·Marina SpörrerBen Fabry

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