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Published on: October 25, 2018
Ocular and generalized myasthenia gravis induced by human acetylcholine receptor γ subunit immunization
Xiaorong Wu1, Erdem Tuzun, Jing Li
1Department of Ophthalmology, Second Xiangya Hospital, Central South University, Changsha, China.
Introduction:
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.
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