Acetylcholine Receptor-Induced Experimental Myasthenia Gravis: What Have We Learned from Animal Models After Three Decades?

  • Fulvio Baggi
  • Carlo Antozzi
  • Chiara Toscani
  • Chiara Cordiglieri


Myasthenia gravis (MG) is an autoimmune disease caused by an immunological response against the acetylcholine receptor (AChR) at the neuromuscular junction. Anti-AChR antibodies induce degradation of the receptor, activation of complement cascade and destruction of the post-synaptic membrane, resulting in a functional reduction of AChR availability. The pathophysiological role of autoantibodies (auto-Abs) and T helper lymphocytes has been studied in the experimental autoimmune MG (EAMG) models. EAMG models have been employed to investigate the factors involved in the development of MG and to suggest new therapies aimed to preventing or modulating the ongoing disease. EAMG can be induced in susceptible mouse and rat strains, which develop clinical symptoms such as muscular weakness and fatigability, mimicking the human disease. Two major types of EAMG can be induced, passive and active EAMG. Passive transfer MG models, involving the injection of auto-Abs, are helpful for studying the role of complement molecules and their regulatory proteins, which can prevent neuromuscular junction degradation. Active models, induced by immunization, are employed for the analysis of antigen-specific immune responses and their modulation in order to improve disease progression. In this review, we will concentrate on the main pathogenic mechanisms of MG, focusing on recent findings on EAMG experimental models.


Myasthenia gravis Autoimmunity Neuroimmunology Experimental model 



Amino acids


Acetylcholine receptor


Torpedo californica AChR




Bone marrow stromal cells


Dendritic cells


Myasthenia gravis


Main immunogenic region


Experimental autoimmune MG


Complete Freund’s adjuvant


Granulocyte–macrophage colony-stimulating factor


Immunoglobulin G type


Membrane attack complex


Neuromuscular junction


Peripheral blood lymphocytes


Pixantrone (BBR2778)


Severe combined immunodeficiency


Transforming growth factor 1-beta


Regulatory T-cell



We regret that the essential work of many investigators and colleagues could not be included in this review due to space constraints.


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Copyright information

© L. Hirszfeld Institute of Immunology and Experimental Therapy, Wroclaw, Poland 2011

Authors and Affiliations

  • Fulvio Baggi
    • 1
  • Carlo Antozzi
    • 1
  • Chiara Toscani
    • 1
  • Chiara Cordiglieri
    • 1
  1. 1.Preclinical Neuroimmunology Laboratory, Neurology IV UnitFondazione Istituto Neurologico “Carlo Besta”MilanItaly

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