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Publication : Dysferlin-deficient muscular dystrophy and innate immune activation.

First Author  Mariano A Year  2013
Journal  FEBS J Volume  280
Issue  17 Pages  4165-76
PubMed ID  23527661 Mgi Jnum  J:214552
Mgi Id  MGI:5603257 Doi  10.1111/febs.12261
Citation  Mariano A, et al. (2013) Dysferlin-deficient muscular dystrophy and innate immune activation. FEBS J 280(17):4165-76
abstractText  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.
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