HAL will be down for maintenance from Friday, June 10 at 4pm through Monday, June 13 at 9am. More information
Skip to Main content Skip to Navigation
Journal articles

Muscle Cells Fix Breaches by Orchestrating a Membrane Repair Ballet

Abstract : Skeletal muscle undergoes many micro-membrane lesions at physiological state. Based on their sizes and magnitude these lesions are repaired via different complexes on a specific spatio-temporal manner. One of the major repair complex is a dysferlin-dependent mechanism. Accordingly, mutations in the DYSF gene encoding dysferlin results in the development of several muscle pathologies called dysferlinopathies, where abnormalities of the membrane repair process have been characterized in patients and animal models. Recent efforts have been deployed to decipher the function of dysferlin, they shed light on its direct implication in sarcolemma resealing after injuries. These discoveries served as a strong ground to design therapeutic approaches for dysferlin-deficient patients. This review detailed the different partners and function of dysferlin and positions the sarcolemma repair in normal and pathological conditions.
Complete list of metadata

Cited literature [106 references]  Display  Hide  Download

Contributor : Marc Bartoli Connect in order to contact the contributor
Submitted on : Monday, February 26, 2018 - 3:47:33 PM
Last modification on : Wednesday, November 3, 2021 - 6:46:35 AM
Long-term archiving on: : Monday, May 28, 2018 - 5:50:34 PM


Muscle Cells Fix Breaches by O...
Files produced by the author(s)




Florian Barthelemy, Aurélia Defour, Nicolas Lévy, Martin Krahn, Marc Bartoli. Muscle Cells Fix Breaches by Orchestrating a Membrane Repair Ballet. Journal of Neuromuscular Diseases, IOS Press, 2018, 5 (1), pp.21 - 28. ⟨10.3233/JND-170251⟩. ⟨hal-01717649⟩



Record views


Files downloads