CROISSANT, Coralie; CARMEILLE, Romain; BREVART, Charlotte; BOUTER, Anthony

doi:10.3390/ijms22105276

CROISSANT, Coralie
Chimie et Biologie des Membranes et des Nanoobjets [CBMN]

CARMEILLE, Romain
Chimie et Biologie des Membranes et des Nanoobjets [CBMN]

BREVART, Charlotte
Chimie et Biologie des Membranes et des Nanoobjets [CBMN]

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Article de revue

Ce document a été publié dans

International journal of molecular sciences. 2021, vol. 22, n° 10, p. 15 p.

Résumé en anglais

Muscular dystrophies constitute a group of genetic disorders that cause weakness and progressive loss of skeletal muscle mass. Among them, Miyoshi muscular dystrophy 1 (MMD1), limb girdle muscular dystrophy type R2 (LGMDR2/2B), and LGMDR12 (2L) are characterized by mutation in gene encoding key membrane-repair protein, which leads to severe dysfunctions in sarcolemma repair. Cell membrane disruption is a physiological event induced by mechanical stress, such as muscle contraction and stretching. Like many eukaryotic cells, muscle fibers possess a protein machinery ensuring fast resealing of damaged plasma membrane. Members of the annexins A (ANXA) family belong to this protein machinery. ANXA are small soluble proteins, twelve in number in humans, which share the property of binding to membranes exposing negatively-charged phospholipids in the presence of calcium (Ca2+). Many ANXA have been reported to participate in membrane repair of varied cell types and species, including human skeletal muscle cells in which they may play a collective role in protection and repair of the sarcolemma. Here, we discuss the participation of ANXA in membrane repair of healthy skeletal muscle cells and how dysregulation of ANXA expression may impact the clinical severity of muscular dystrophies.< Réduire

Mots clés en anglais

annexins

muscular dystrophy

membrane repair

skeletal muscle

genetic modifiers

LGMD

FSHD

DMD

URI

https://oskar-bordeaux.fr/handle/20.500.12278/95015

DOI

http://dx.doi.org/10.3390/ijms22105276

Unités de recherche

CBMN : Chimie & de Biologie des Membranes & des Nano-objets - UMR 5248

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Annexins and Membrane Repair Dysfunctions in Muscular Dystrophies

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Résumé en anglais

Mots clés en anglais

URI

DOI

Unités de recherche