title: Cell surface complement regulators moderate experimental myasthenia gravis pathology.
abstract: BACKGROUND
  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.
  
  
  CONCLUSIONS
  NMJs of CD59 and DAF mice are protected from complement-mediated injury of passive EAMG.
authors.affiliation: Department of Pharmacology and Physiology, George Washington University, Washington, DC, USA. lkusner@gwu.edu
authors.forename: Linda L
authors.initials: LL
authors.lastname: Kusner
authors.forename: Jose A
authors.initials: JA
authors.lastname: Halperin
authors.forename: Henry J
authors.initials: HJ
authors.lastname: Kaminski
mesh_terms: D000818:Animals; D018957:CD59 Antigens / Q000276:immunology*; D002462:Cell Membrane / Q000276:immunology* / Q000473:pathology; D003165:Complement System Proteins / Q000276:immunology*; D051379:Mice; D008822:Mice, Transgenic; D020720:Myasthenia Gravis, Autoimmune, Experimental / Q000276:immunology* / Q000473:pathology; D009469:Neuromuscular Junction / Q000276:immunology* / Q000473:pathology; D011950:Receptors, Cholinergic / Q000276:immunology
chemical_list: D018957:CD59 Antigens; D011950:Receptors, Cholinergic; D003165:Complement System Proteins