%0 Journal Article
%T Muscle membrane repair and inflammatory attack in dysferlinopathy
%A Renzhi Han
%J Skeletal Muscle
%D 2011
%I BioMed Central
%R 10.1186/2044-5040-1-10
%X Damage to the plasma membrane induces entry of toxic agents such as calcium and oxidants into the cells, releases intracellular molecules producing inflammatory responses, and threatens the afflicted cells with an immediate cell death. Recent studies reveal a rapid membrane repair response that is conserved in many different types of cells to restore the plasma membrane integrity and enables the cells to survive following a limited level of membrane disruptions [1-7]. Defects in this process can result in pathological complications in a number of different tissues, particularly the skeletal muscle and heart [8-11]. Moreover, continuous release of intracellular contents from cells with defective membrane repair exposes "danger" signals to the immune system of the host and causes further tissue damage [12-14].It is known that the membrane repair process requires intracellular vesicles [15] which deliver excess membrane to form a "membrane patch" through Ca2+-triggered vesicular exocytosis [16,17] similar to neurotransmitter release [18] (Figure 1). The intracellular vesicles are initially transported to the damage site via the sequential actions of the motor proteins including kinesin and non-muscle myosin IIA and IIB in sea urchin eggs and several cell lines such as 3T3 fibroblasts and COS-7 [19,20]. Myosin IIB is required for the exocytosis and membrane repair itself while myosin IIA is required in facilitation of cell membrane repair at repeated wounds [20]. However, the involvement of these motor proteins in muscle membrane repair has not been determined. Recently, Mitsugumin 53 (MG53), a muscle-specific tripartite motif family protein (TRIM72), has also come into play in vesicle translocation during muscle membrane repair [21-26]. MG53 is observed to rapidly accumulate at the damage site following membrane disruption. Genetic ablation of MG53 results in a late-onset progressive skeletal myopathy [24] and increases susceptibility to ischemia/reperfusion-induced my
%U http://www.skeletalmusclejournal.com/content/1/1/10