Dysferlin-deficient muscular dystrophy and innate immune activation Journal Article

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Authors: Mariano, A.; Henning, A.; Han, R.
Article Title: Dysferlin-deficient muscular dystrophy and innate immune activation
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.
Journal Title: The FEBS journal
Volume: 280
Issue: 17
ISSN: 1742-4658; 1742-464X
Publisher: FEBS  
Journal Place: England
Date Published: 2013
Start Page: 4165
End Page: 4176
Language: eng
DOI/URL:

10.1111/febs.12261

10.1111/febs.12261
Notes: CI: (c) 2013 The Authors Journal compilation (c) 2013; JID: 101229646; OTO: NOTNLM; 2013/01/02 [received]; 2013/03/06 [revised]; 2013/03/20 [accepted]; 2013/04/22 [aheadofprint]; ppublish