4.5 Article

Dysferlin and muscle membrane repair

Journal

CURRENT OPINION IN CELL BIOLOGY
Volume 19, Issue 4, Pages 409-416

Publisher

CURRENT BIOLOGY LTD
DOI: 10.1016/j.ceb.2007.07.001

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Funding

  1. NIAMS NIH HHS [R01 AR051199-04, R01 AR051199] Funding Source: Medline
  2. NINDS NIH HHS [U54 NS053672, 1 U54 NS053672, U54 NS053672-03] Funding Source: Medline

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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.

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