Journal
CELL BIOLOGY INTERNATIONAL
Volume 39, Issue 4, Pages 361-363Publisher
WILEY
DOI: 10.1002/cbin.10384
Keywords
intermediate filament proteins; myoblasts; myotubes; mechano-transduction
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Funding
- Staedtler-Stiftung [DS/eh/11/14]
- Deutsche Forschungsgemeinschaft (DFG) [FOR1228]
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Myofibrillar myopathies (MFMs) are a group of sporadic and hereditary skeletal muscle diseases, which lead to severe physical disability and premature death. Most MFMs are caused by mutations in genes encoding desmin, plectin, VCP, filamin C, BAG3, FHL-1, B-crystallin, DNAJB6, myotilin, and ZASP. Biomechanical studies on primary human myoblasts carrying desmin and plectin mutations showed increased stiffness and reduced mechanical stress tolerance i.e., higher mechanical vulnerability compared to control cells. Higher stiffness of mutant cells may lead to higher intracellular stress at physiologic stretch and shear deformation, which in turn could trigger muscle fiber degeneration.
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