Animal models of myasthenia gravis: utility and limitations

Renato Mantegazza, Chiara Cordiglieri, Alessandra Consonni, Fulvio Baggi Neurology IV Unit, Neuroimmunology and Neuromuscular Disorders, Foundation IRCCS Neurological Institute “Carlo Besta”, Milan, Italy Abstract: Myasthenia gravis (MG) is a chronic autoimmune disease caused by the immune attack of the neuromuscular junction. Antibodies directed against the acetylcholine receptor (AChR) induce receptor degradation, complement cascade activation, and postsynaptic membrane destruction, resulting in functional reduction in AChR availability. Besides anti-AChR antibodies, other autoantibodies are known to play pathogenic roles in MG. The experimental autoimmune MG (EAMG) models have been of great help over the years in understanding the pathophysiological role of specific autoantibodies and T helper lymphocytes and in suggesting new therapies for prevention and modulation of the ongoing disease. EAMG can be induced in mice and rats of susceptible strains that show clinical symptoms mimicking the human disease. EAMG models are helpful for studying both the muscle and the immune compartments to evaluate new treatment perspectives. In this review, we concentrate on recent findings on EAMG models, focusing on their utility and limitations. Keywords: myasthenia gravis, autoimmunity, neuroimmunology, AChR