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

WU X., Tuzun E., LI J., XIAO T., SAINI S. S., QI H., ...More

MUSCLE & NERVE, vol.45, no.2, pp.209-216, 2012 (SCI-Expanded) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 45 Issue: 2
  • Publication Date: 2012
  • Doi Number: 10.1002/mus.22273
  • Journal Name: MUSCLE & NERVE
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Page Numbers: pp.209-216
  • Keywords: acetylcholine receptor ? subunit, autoimmune disease, extraocular muscles, myasthenia gravis, ocular myasthenia gravis, HLA TRANSGENIC MICE, EXTRAOCULAR-MUSCLE, GENE-EXPRESSION, GAMMA-SUBUNIT, SKELETAL-MUSCLE, ALPHA-SUBUNIT, MOUSE MODEL, PATHOGENESIS, INDUCTION, MECHANISM
  • Istanbul University Affiliated: Yes


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) a subunit. Because the fetal AChR ? subunit is expressed in adult extraocular muscles, we anticipated that ? subunit immunization would generate an immune response to mouse AChR and induce MG in mice. Results: H-AChR ? 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 ? subunit. Muscle Nerve, 2012