Journal
CURRENT OPINION IN CELL BIOLOGY
Volume 32, Issue -, Pages 113-120Publisher
CURRENT BIOLOGY LTD
DOI: 10.1016/j.ceb.2015.01.004
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Funding
- COST [BM1002]
- Greek Secretariat for RD [PENED 01ED371, EPAN YB-22, PEP ATT-39, ESPA SYN 965]
- [NIH-AR39617]
- NATIONAL INSTITUTE OF ARTHRITIS AND MUSCULOSKELETAL AND SKIN DISEASES [R01AR039617] Funding Source: NIH RePORTER
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Maintenance of the highly organized striated muscle tissue requires a cell-wide dynamic network that through interactions with all vital cell structures, provides an effective mechanochemical integrator of morphology and function, absolutely necessary for intra-cellular and intercellular coordination of all muscle functions. A good candidate for such a system is the desmin intermediate filament cytoskeletal network. Human desmin mutations and post-translational modifications cause disturbance of this network, thus leading to loss of function of both desmin and its binding partners, as well as potential toxic effects of the formed aggregates. Both loss of normal function and gain of toxic function are linked to mitochondrial defects, cardiomyocyte death, muscle degeneration and development of skeletal myopathy and cardiomyopathy.
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