Experimental models of myasthenia gravis: lessons in autoimmunity and progress toward better forms of treatment.
The nicotinic acetylcholine receptor (AChR) is a large membrane protein found in muscle cells. It is involved in the transformation of acetylcholine packets into a membrane depolarization, which thereby leads to a muscle twitch. This large, complex molecule is the target of the autoimmune attack in myasthenia gravis, and much has been learned in the past decade about myasthenia by the induction of autoimmunity to AChR in experimental animals. Experimental autoimmune myasthenia gravis (EAMG) has been produced in a variety of animals by immunization with AChR or AChR-like material, or by the passive transfer of anti-AChR antibodies or lymphocytes from afflicted animals into normal animals. EAMG is a remarkably faithful model of human myasthenia and has provided much information about how the immune response to AChR progresses and how weakness and damage to the neuromuscular junction ensure. EAMG has also allowed the development of a number of revolutionary forms of treatment in which only the abnormal response to AChR is restrained, and other necessary immune functions are left intact. These advances in treatment are not far from being tested in human myasthenia gravis. The experience gained in applying these concepts in EAMG and human myasthenia will be helpful in developing similar forms of treatment for other autoimmune diseases.
D000818:Animals; D000888:Antibodies, Anti-Idiotypic / Q000276:immunology; D004195:Disease Models, Animal; D007111:Immunity, Cellular; D007114:Immunization; D007116:Immunization, Passive; D007130:Immunoglobulin Idiotypes / Q000276:immunology; D009157:Myasthenia Gravis / Q000276:immunology* / Q000628:therapy; D011978:Receptors, Nicotinic / Q000276:immunology*
D000888:Antibodies, Anti-Idiotypic; D007130:Immunoglobulin Idiotypes; D011978:Receptors, Nicotinic