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Cell surface complement regulators moderate experimental myasthenia gravis pathology
Author(s) -
Kusner Linda L.,
Halperin Jose A.,
Kaminski Henry J.
Publication year - 2013
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.23448
Subject(s) - myasthenia gravis , acetylcholine receptor , neuromuscular junction , muscle weakness , complement system , weakness , acetylcholine , medicine , cd59 , immunology , pathology , biology , receptor , antibody , anatomy , neuroscience
Intrinsic mouse complement regulators influence the severity of passively induced experimental acquired myasthenia gravis (EAMG). To assess the potential influence of CD59b in the absence of CD59a background, we used the mCD59ab −/− mouse model to re‐evaluate mCD59 in protecting the neuromuscular junction (NMJ). Methods: EAMG was induced with monoclonal antibody to the acetylcholine receptor (AChR) in Daf1 −/− , CD59ab −/− , Daf1 −/− CD59ab −/− , and wild‐type C57Bl/6 mice. Animals were monitored throughout the experiment. Diaphragms were analyzed for NMJ injury. Results: Daf1 −/− CD59ab −/− mice required euthanasia 24 hours after disease induction because of severe weakness. Histological assessment demonstrated reduced AChR density, simplification of synaptic folds, and disrupted mitochondria. CD59ab‐deficient mice demonstrated mild weakness and reduction in weight after 24 hours. In contrast, Daf1 −/− had more severe weakness at 60 hours. The NMJ of EAMG‐induced Daf1 −/− and CD59ab −/− mice demonstrated similar AChR density. Conclusion: NMJs of CD59 and DAF mice are protected from complement‐mediated injury of passive EAMG. Muscle Nerve, 2013