Immunoregulation in Experimental Autoimmune Myasthenia Gravis—about T Cells, Antibodies, and Endplates
- 1 September 2003
- journal article
- research article
- Published by Wiley in Annals of the New York Academy of Sciences
- Vol. 998 (1), 308-317
- https://doi.org/10.1196/annals.1254.033
Abstract
Experimental autoimmune myasthenia gravis (EAMG) can be induced in a large number of animal species by active immunization (AI) AChR, by passive transfer (PT) of anti-AChR antibodies, by autologous bone marrow transplantation and cyclosporin (BMT-Cy), or spontaneously. Depending on the model used, different immunological mechanisms are operational. In the AI model, the T cell is pivotal in directing the anti-AChR antibody production towards pathogenic, that is, cross-linking and complement-fixing antibodies. Injection of anti-AChR antibodies alone suffices to induce EAMG, excluding the role of specific cell-mediated immune responses in the effector phase of the disease. Aged animals are resistant to the induction of AI and PT EAMG. This resistance is localized at the postsynaptic membrane containing more AChR-anchoring proteins, including S-laminin and rapsyn in aged animals. In BMT-CyA EAMG, a dysregulation of the immune system in the absence of immunization is capable of inducing myasthenia. The role of these animal models in relation to pathogenesis and immunotherapy is discussed.Keywords
This publication has 36 references indexed in Scilit:
- Characterization of Human Anti-acetylcholine Receptor Monoclonal Autoantibodies from the Peripheral Blood of a Myasthenia Gravis Patient Using Combinatorial LibrariesClinical Immunology, 2000
- Animal Models of Myasthenia GravisClinical Immunology, 2000
- Anatomy of the antigenic structure of a large memberane autoantigen, the muscle‐type nicotinic acetylcholine receptorImmunological Reviews, 1998
- Maintenance of Acetylcholine Receptor Number by Neuregulins at the Neuromuscular Junction in VivoScience, 1997
- Myasthenia gravis-like syndrome induced by expression of interferon gamma in the neuromuscular junction.The Journal of Experimental Medicine, 1995
- Acetylcholine receptor and myogenic factor gene expression in Torpedo embryonic developmentNeuroReport, 1994
- Transplantation of thymic autoimmune microenvironment to severe combined immunodeficiency mice. A new model of myasthenia gravis.JCI Insight, 1992
- Acetylcholine receptor-reactive antibodies in experimental autoimmune myasthenia gravis differing in disease-causing potential: Subsetting of serum antibodies by preparative isoelectric focusingClinical Immunology and Immunopathology, 1992
- Acetylcholine Receptor Antibody in Myasthenia Gravis: Purification and CharacterizationScandinavian Journal of Immunology, 1978
- Autoimmune Response to Acetylcholine ReceptorScience, 1973