Ocular and generalized myasthenia gravis induced by human acetylcholine receptor γ subunit immunization

Xiaorong Wu, Erdem Tuzun, Jing Li, Tianlin Xiao, Shamsher S. Saini, Huibin Qi, Windy Allman, Premkumar Christadoss

Research output: Contribution to journalArticle

17 Scopus citations

Abstract

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.

Original languageEnglish (US)
Pages (from-to)209-216
Number of pages8
JournalMuscle and Nerve
Volume45
Issue number2
DOIs
StatePublished - Feb 1 2012

Keywords

  • Acetylcholine receptor γ subunit
  • Autoimmune disease
  • Extraocular muscles
  • Myasthenia gravis
  • Ocular myasthenia gravis

ASJC Scopus subject areas

  • Physiology
  • Clinical Neurology
  • Cellular and Molecular Neuroscience
  • Physiology (medical)

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  • Cite this

    Wu, X., Tuzun, E., Li, J., Xiao, T., Saini, S. S., Qi, H., Allman, W., & Christadoss, P. (2012). Ocular and generalized myasthenia gravis induced by human acetylcholine receptor γ subunit immunization. Muscle and Nerve, 45(2), 209-216. https://doi.org/10.1002/mus.22273