Novel animal models of acetylcholine receptor antibody-related myasthenia gravis

Erdem Tüzün, Windy Allman, Canan Ulusoy, Huan Yang, Premkumar Christadoss

Research output: Contribution to journalArticle

8 Scopus citations

Abstract

Experimental autoimmune myasthenia gravis (EAMG) in mice has been used to unravel the pathogenic mechanisms and to be used as a preclinical model of myasthenia gravis (MG). Induction of predominantly ocular EAMG in HLA-DQ8 transgenic mice immunized with acetylcholine receptor (AChR) subunits demonstrated the importance of nonconformationally expressed AChR subunits in extraocular muscle involvement. Wild-type (WT) and CD4+ T cell knockout (KO) C57BL/6 mice developed EAMG upon immunization with AChR in incomplete Freund's adjuvant plus lipopolysaccharide. AChR-specific IgG2+ B cell frequencies, estimated by Alexa-conjugated AChR, and AChR-reactive IgG2b levels significantly correlated with the clinical grades of EAMG in WT mice. CD4+ T cell-deficient EAMG mice exhibited AChR antibodies mainly of the IgG2b isotype, emphasizing T helper-independent B cell activation pathways in EAMG induction. These novel EAMG models have suggested that diverse immunopathological mechanisms might contribute to EAMG or MG pathogenesis.

Original languageEnglish (US)
Pages (from-to)133-139
Number of pages7
JournalAnnals of the New York Academy of Sciences
Volume1274
Issue number1
DOIs
StatePublished - Dec 2012

Keywords

  • Autoimmunity
  • Experimental autoimmune myasthenia gravis
  • Lipopolysaccharide
  • Myasthenia gravis

ASJC Scopus subject areas

  • Neuroscience(all)
  • Biochemistry, Genetics and Molecular Biology(all)
  • History and Philosophy of Science

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