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

Dysferlin-deficient muscular dystrophy and innate immune activation.

Author information

1
Department of Cell and Molecular Physiology, Loyola University Chicago Health Science Division, Maywood, IL 60153, USA.

Abstract

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

PMID:
23527661
DOI:
10.1111/febs.12261
[Indexed for MEDLINE]
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