Dysferlin and muscle membrane repair
WebHere, we have studied the mechanism of AnxA2-mediated muscle cell membrane repair in cultured muscle cells. We find that injury-triggered increase in cytosolic calcium causes … WebJun 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 …
Dysferlin and muscle membrane repair
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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. … WebApr 1, 2004 · Figure 3 Dysferlin-mediated membrane repair model. In normal muscle fiber, dysferlin is localized at the plasma membrane (where it interacts with annexin A1 and A2) and cytoplasmic vesicles (a). A …
WebJun 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. Membrane resealing is a function conserved by most cells and is mediated by a mechanism closely resembling regulated, Ca2+-dependent exocytosis. WebDysferlin is a membrane-associated protein implicated in sarcolemmal repair and also linked to other membrane functions including the maintenance of transverse tubules in …
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 … WebDec 31, 2016 · MG53, annexins, and Eps15 homology domain-containing proteins interact with dysferlin to form a membrane repair complex and similarly have roles in membrane trafficking in muscle. These molecular ...
Webism [6 ]. Dysferlin-null muscle fibers fail to exclude the dye entry even in the presence of Ca2+ (Figure 1), strongly suggestingtheCa2+-dependentmembrane repair requires dysferlin [6 ]. Loss of dysferlin-mediated mem-brane repair results in progressive myonecrosis [6 ,10 ]. In this review, we discuss recent advances in our un-derstanding of ...
WebDysferlin also known as dystrophy-associated fer-1-like protein is a protein that in humans is encoded by the DYSF gene.. Dysferlin is linked with stabilization of calcium signaling and membrane repair. A defect in the DYSF gene, located on chromosome 2p12-14, results in several types of muscular dystrophy; including Miyoshi myopathy (MM), Limb-girdle … raymond and hayley stiehlWebApr 1, 2004 · Defective plasma membrane repair in dysferlin-null muscle cellsThe plasma membrane of a cell provides a physical barrier between extracellular and intracellular … simplicity 9691WebSep 1, 2007 · This is consistent with results from our laboratory showing that membrane repair after eccentric injury of skeletal muscle in vivo is not impaired by the absence of dysferlin (Roche et al., 2010 ... simplicity 9695WebMany muscular dystrophies arise from enhanced muscle degeneration, but one muscular dystrophy subtype has now been shown to arise from defective muscle membrane repair. Mutations in the gene encoding dysferlin cause muscular dystrophy, and recent work has demonstrated a role for this protein in resealing muscle membrane tears. Thus, two … raymond and heather ziferWebMar 1, 2011 · Repair of plasma membrane tears is an important normal physiological process that enables the cells to survive a variety of physiological and pathological membrane lesions. Dysferlin was the … raymond and flanigan locationWebApr 1, 2004 · Membrane-repair model. In the light of the recent findings, a model describing dysferlin-mediated membrane repair in the skeletal muscle cells is presented in Figure 3. The model states that a membrane disruption causes influx of extracellular calcium into the muscle fiber and creates a transient zone of high calcium around the injury site. raymond and grahamWebNov 11, 2016 · Dysferlin deficit in mdx mice further compromises myofiber cell membrane repair and enhances the muscle pathology at an asymptomatic age for dysferlin-deficient mice. raymond and fleming furniture