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Updated: May 13, 2026

Immunolabelling Myofiber Degeneration in Muscle Biopsies
Published on: December 5, 2019
Dysferlin-deficient muscular dystrophy and innate immune activation
Andrew Mariano1, Audrey Henning, Renzhi Han
1Department 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.
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