Dysferlin and muscle membrane repair
Dysferlin and muscle membrane repair
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DOI：
10.1016/j.ceb.2007.07.001
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发表时间：
2007-08-01
影响因子：
7.5
通讯作者：
Campbell, Kevin P.
中科院分区：
文献类型：
--
作者：
Han, Renzhi;Campbell, Kevin P.
修复膜损伤的能力在真核细胞中是保守的，并且是细胞在各种生理和病理膜破坏中存活所必需的。膜修复是由快速Ca2+触发的各种细胞内囊泡（如溶酶体和增大体）的胞外分泌介导的，这导致形成一个膜补丁，重新密封膜损伤。最近的研究结果表明，dysferlin在肌肉的修复过程中起着至关重要的作用，可能是作为触发囊泡融合的Ca2+传感器。膜修复的重要性被遗传性疾病异常铁蛋白病所强调，其中主要缺陷是由于异常铁蛋白缺乏导致Ca2+调节的膜修复丧失。未来对异草素及其相互作用伙伴的研究将增强对这一重要过程的理解，并为潜在的治疗提供新的途径。
The ability to repair membrane damage is conserved across eukaryotic cells and is necessary for the cells to survive a variety of physiological and pathological membrane disruptions. Membrane repair is mediated by rapid Ca2+-triggered exocytosis of various intracellular vesicles, such as lysosomes and enlargeosomes, which lead to the formation of a membrane patch that reseals the membrane lesion. Recent findings suggest a crucial role for dysferlin in this repair process in muscle, possibly as a Ca2+ sensor that triggers vesicle fusion. The importance of membrane repair is highlighted by the genetic disease, dysferlinopathy, in which the primary defect is the loss of Ca2+ -regulated membrane repair due to dysferlin deficiency. Future research on dysferlin and its interacting partners Will enhance the understanding of this important process and provide novel avenues to potential therapies.