Muscle Cells Fix Breaches by Orchestrating a Membrane Repair Ballet

Journal of Neuromuscular Diseases
Florian BarthélémyMarc Bartoli

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.

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Citations

Jan 27, 2019·Expert Review of Proteomics·Sandra MurphyKay Ohlendieck
Feb 1, 2020·The Journal of Physiology·Laura GerwinStephan Kröger
Feb 29, 2020·Cells·Anna V Bulankina, Sven Thoms
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Jan 8, 2021·Skeletal Muscle·Stephan Kröger, Bridgette Watkins
Apr 15, 2021·BMC Biology·Dustin A AmmendoliaJohn H Brumell
May 15, 2021·Archives of Gynecology and Obstetrics·Li-Sha PengYang Zou
Sep 5, 2021·FASEB Journal : Official Publication of the Federation of American Societies for Experimental Biology·Kevin A MurachCharlotte A Peterson

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Methods Mentioned

BETA
GTPases
electronic microscopy

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