Autoimmune diseases against cell surface receptors: myasthenia gravis, a prototype anti-receptor disease.
Autoimmune diseases against cell surface receptors are the result of a mainly antibody-mediated attack on membrane receptors. This results in a hypofunction of the target organ; occasionally antibodies can exert an agonist effect, e.g. in Graves' disease. Myasthenia gravis (MG) is an autoimmune disease of the neuromuscular junction associated with a plethora of other diseases, mainly autoimmune diseases. Antibodies against the acetylcholine receptor (AChR) reduce the number of receptors necessary for efficient neuromuscular transmission. The effector mechanisms of MG can be studied elegantly in an experimental animal model in rodents immunized with AChR or injected with antibodies against AChR. The thymus is thought to play a central role in the induction of MG. Microscopic analysis of these thymuses revealed a follicular hyperplasia of the medulla or a lympho-epithelial thymoma. Thymectomy results in clinical improvement along with a decline in anti-AChR antibody titres. Additional therapeutic measures include anticholinesterase drugs, immunosuppression and plasmapheresis.
Department of Immunology, University of Limburg, Maastricht, Netherlands.
M H
MH
De Baets
D000818:Animals; D001323:Autoantibodies / Q000276:immunology*; D001327:Autoimmune Diseases / Q000175:diagnosis / Q000453:epidemiology / Q000276:immunology* / Q000628:therapy; D004195:Disease Models, Animal; D005260:Female; D006801:Humans; D008297:Male; D009157:Myasthenia Gravis / Q000175:diagnosis / Q000453:epidemiology / Q000276:immunology* / Q000628:therapy; D011956:Receptors, Cell Surface / Q000276:immunology*; D013934:Thymectomy; D013950:Thymus Gland / Q000276:immunology
D001323:Autoantibodies; D011956:Receptors, Cell Surface