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Review
. 2018;5(1):21-28.
doi: 10.3233/JND-170251.

Muscle Cells Fix Breaches by Orchestrating a Membrane Repair Ballet

Florian Barthélémy  1   2 , Aurélia Defour  3 , Nicolas Lévy  3 , Martin Krahn  3 , Marc Bartoli  3
Affiliations
Review

Muscle Cells Fix Breaches by Orchestrating a Membrane Repair Ballet

Florian Barthélémy et al. J Neuromuscul Dis. 2018.

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.

Keywords: Muscle; dysferlin; dystrophy; membrane; myopathy.

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Figures

Fig.1
Fig.1
Membrane repair in muscle cell, Schematic representation of the different events from an emergency patch to a functional recovery in muscle cell after mechanic lesion. See text for description.
See this image and copyright information in PMC

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Supplementary concepts