Dysferlin-deficient muscular dystrophy and innate immune activation

被引：28
作者：
Mariano, Andrew [1 ]
Henning, Audrey [1 ]
Han, Renzhi [1 ]
机构：
[1] Loyola Univ Chicago, Dept Cell & Mol Physiol, Div Hlth Sci, Maywood, IL 60153 USA
来源：
FEBS JOURNAL | 2013年 / 280卷 / 17期
关键词：
dysferlin; inflammation; innate immune system; membrane repair; muscular dystrophy; CELL-MEMBRANE REPAIR; LINKS OXIDATIVE STRESS; TOLL-LIKE RECEPTORS; C2 DOMAINS REVEALS; SKELETAL-MUSCLE; NALP3; INFLAMMASOME; UP-REGULATION; T-TUBULE; COMPLEMENT; MYOPATHY;
D O I：
10.1111/febs.12261
中图分类号：
Q5 [生物化学]; Q7 [分子生物学];
学科分类号：
071010 ; 081704 ;
摘要：
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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收藏
页码：4165 / 4176
页数：12
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