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Ocular and generalized myasthenia gravis induced by human acetylcholine receptor γ subunit immunization
Author(s) -
Wu Xiaorong,
Tuzun Erdem,
Li Jing,
Xiao Tianlin,
Saini Shamsher S.,
Qi Huibin,
Allman Windy,
Christadoss Premkumar
Publication year - 2012
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.22273
Subject(s) - myasthenia gravis , acetylcholine receptor , neuromuscular junction , protein subunit , downregulation and upregulation , extraocular muscles , ocular myasthenia , immune system , immunology , immunization , receptor , endocrinology , biology , medicine , anatomy , neuroscience , gene , biochemistry
: 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. Conclusion : Our findings indicate that oMG may be induced by immunity to the AChR γ subunit. Muscle Nerve, 2012