Dysferlin and muscle membrane repair
Webestablishing the function of dysferlin in muscle membrane repair [1,2,3,15], little is known about how dysferlin exerts its function. Dysferlin is a 230 kDa type II transmembrane protein, belonging to the ferlin-1-like protein family [9,16]. All ferlin-1-like proteins contain multiple C2 domains that were known to possess the WebDysferlin is a membrane-associated protein implicated in sarcolemmal repair and also linked to other membrane functions including the maintenance of transverse tubules in muscle. MG53, annexins, and Eps15-homology domain (EHD)-containing proteins interact with dysferlin to form a membrane repair complex and similarly have roles in …
Dysferlin and muscle membrane repair
Did you know?
WebApr 1, 2004 · Defective plasma membrane repair in dysferlin-null muscle cellsThe plasma membrane of a cell provides a physical barrier between extracellular and intracellular … WebThe DYSF gene provides instructions for making a protein called dysferlin. This protein is found in the thin membrane called the sarcolemma that surrounds muscle fibers. …
WebMar 3, 2024 · Dysferlin is a membrane protein that facilitates lysosomal vesicle fusion with the sarcolemma; the absence of dysferlin is associated with the inability to repair muscle membranes after exercise-induced injury. An early step in normal muscle repair is the fusion of lysosomal vesicles with the sarcolemma, allowing the release of ASM. WebApr 1, 2011 · The current paradigm for muscle membrane resealing centers on a role for dysferlin and the annexins in calcium-activated vesicle fusion for membrane patch repair. Mitsugumin 53 may act upstream of dysferlin and function independently of calcium, acting as an “oxidation sensor” that is activated by a change in the intracellular redox state ...
WebMay 8, 2003 · Unlike dysferlin-null fibres, membrane repair assays conducted on muscle fibres in mdx mice showed efficient membrane resealing in the presence of Ca 2+ … WebJan 18, 2024 · Dysferlin is a Ca2+-activated lipid binding protein implicated in muscle membrane repair. Recessive variants in DYSF result in dysferlinopathy, a progressive muscular dystrophy. We showed …
WebAdditionally, activation of the dysferlin-AMPK complex markedly improved membrane repair (line 107; Figures 6A–6E) and the muscle phenotype in zebrafish and SJL/J mice , exhibiting with residual expression of dysferlin. Therefore, utilization of the protein-protein interaction of AMPK with dysferlin for developing a therapeutic strategy in ... bird in hand westhay menuWebJun 5, 2007 · Dysferlin is the first identified member of a putative muscle-specific repair complex that permits rapid resealing of membranes disrupted by mechanical stress. … bird in hand winery adelaideWebAug 19, 2024 · Here, we have studied the mechanism of AnxA2-mediated muscle cell membrane repair in cultured muscle cells. We find that injury-triggered increase in … damarco williamsWebAug 1, 2003 · In support of a role for dysferlin in membrane fusion, electron micrographs from dysferlin-mutant muscle showed an increased number of sub-sarcolemmal … bird in hand witney lunch menuWebJun 5, 2009 · Defective membrane repair can contribute to the progression of muscular dystrophy. Although mutations in caveolin-3 (Cav3) and dysferlin are linked to muscular dystrophy in human patients, the molecular mechanism underlying the functional interplay between Cav3 and dysferlin in membrane repair of muscle physiology and disease … damaraland camp by wilderness safarisWebMembrane repair is a critical mechanism for cell survival, and well-characterized proteins such as MG53 and dysferlin have been implicated in the resealing of human and mouse muscle plasma membrane. MG53 and dysferlin accumulate at the site of membrane damage in muscle fibers ( Bansal et al., 2003 ; Cai et al., 2009 ; Marg et al., 2012 ). bird in hand witney oxfordshireWebMany muscular dystrophies arise from enhanced muscle degeneration, but one muscular dystrophy subtype has now been shown to arise from defective muscle membrane … damarcus williams