Dysferlin-deficient muscular dystrophy and innate immune activation

FEBS J. 2013 Sep;280(17):4165-76. doi: 10.1111/febs.12261. Epub 2013 Apr 22.


Cells encounter many physical, chemical and biological stresses that perturb plasma membrane integrity, warranting an immediate membrane repair response to regain cell homeostasis. Failure to respond properly to such perturbation leads to individual cell death, which may also produce systemic influence by triggering sterile immunological responses. In this review, we discuss recent progress on understanding the mechanisms underlying muscle cell membrane repair and the potential mediators of innate immune activation when the membrane repair system is defective, specifically focusing on pathology associated with dysferlin deficiency.

Keywords: dysferlin; inflammation; innate immune system; membrane repair; muscular dystrophy.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • Cell Membrane / immunology*
  • Cell Membrane / metabolism
  • Dysferlin
  • Immunity, Innate*
  • Membrane Proteins / physiology*
  • Mice
  • Mice, Knockout
  • Muscle, Skeletal / cytology
  • Muscle, Skeletal / immunology*
  • Muscle, Skeletal / metabolism
  • Muscular Dystrophies / immunology*
  • Muscular Dystrophies / metabolism
  • Muscular Dystrophies / pathology


  • Dysf protein, mouse
  • Dysferlin
  • Membrane Proteins