title: Ocular and generalized myasthenia gravis induced by human acetylcholine receptor γ subunit immunization.
abstract: BACKGROUND
  HLA-DQ8 transgenic mice develop ocular myasthenia gravis (oMG), which then progresses to generalized MG (gMG) when immunized with the human acetylcholine receptor (H-AChR) α subunit. Because the fetal AChR γ subunit is expressed in adult extraocular muscles, we anticipated that γ subunit immunization would generate an immune response to mouse AChR and induce MG in mice.
  
  
  RESULTS
  H-AChR γ subunit immunization in HLA-DQ8 mice induced an autoimmune response to mouse AChR and led to the destruction of AChR in the neuromuscular junction (NMJ) by anti-AChR antibody and complement activation, and it triggered upregulation of AChR gene transcription.
  
  
  CONCLUSIONS
  Our findings indicate that oMG may be induced by immunity to the AChR γ subunit.
authors.affiliation: Department of Ophthalmology, Second Xiangya Hospital, Central South University, Changsha, China.
authors.forename: Xiaorong
authors.initials: X
authors.lastname: Wu
authors.forename: Erdem
authors.initials: E
authors.lastname: Tuzun
authors.forename: Jing
authors.initials: J
authors.lastname: Li
authors.forename: Tianlin
authors.initials: T
authors.lastname: Xiao
authors.forename: Shamsher S
authors.initials: SS
authors.lastname: Saini
authors.forename: Huibin
authors.initials: H
authors.lastname: Qi
authors.forename: Windy
authors.initials: W
authors.lastname: Allman
authors.forename: Premkumar
authors.initials: P
authors.lastname: Christadoss
mesh_terms: D000818:Animals; D000888:Antibodies, Anti-Idiotypic / Q000097:blood / Q000276:immunology; D029681:Arabidopsis Proteins / Q000378:metabolism; D049109:Cell Proliferation; D003176:Complement C3 / Q000378:metabolism; D004195:Disease Models, Animal; D004797:Enzyme-Linked Immunosorbent Assay; D006683:HLA-DQ Antigens / Q000235:genetics; D006801:Humans; D007114:Immunization / Q000009:adverse effects*; D019751:Intramolecular Transferases / Q000378:metabolism; D008213:Lymphocyte Activation / Q000276:immunology; D051379:Mice; D008822:Mice, Transgenic; D053580:Muscle Strength / Q000235:genetics; D020720:Myasthenia Gravis, Autoimmune, Experimental / Q000097:blood / Q000209:etiology* / Q000235:genetics / Q000473:pathology; D009469:Neuromuscular Junction / Q000276:immunology; D015835:Ocular Motility Disorders / Q000209:etiology / Q000276:immunology; D021122:Protein Subunits / Q000235:genetics / Q000378:metabolism; D011978:Receptors, Nicotinic / Q000276:immunology*
chemical_list: D000888:Antibodies, Anti-Idiotypic; D029681:Arabidopsis Proteins; C539275:CHRNG protein, human; D003176:Complement C3; D006683:HLA-DQ Antigens; C109756:HLA-DQ8 antigen; D021122:Protein Subunits; D011978:Receptors, Nicotinic; D019751:Intramolecular Transferases; C568454:marneral synthase, Arabidopsis