Acetylcholine receptors and myasthenia gravis.
Recent years have seen considerable progress in understanding the nature of the molecular events involved in neuromuscular transmission. The acetylcholine receptor (AChR) has been purified to homogeneity and acetylcholine-induced ion transport has been reconstituted by incorporation of pure AChR into artificial membranes. Immunization against purified AChR induces a condition, clinically and physiologically similar to the human disease myasthenia gravis, which is due to circulating anti-AChR antibodies. This model, experimental autoimmune myasthenia gravis, is proving useful for investigating the role of genetic factors in determining the immune response to AChRs and for testing various experimental approaches to specific treatment. Myasthenia gravis is an autoimmune disease in which there is loss of acetylcholine receptors at the neuromuscular junction. Anti-AChR antibodies can be detected in the majority of patients and they cause loss of AChR by a variety of mechanisms. Anti-AChR antibody is heterogeneous and not restricted in idiotype. The role of the thymus in MG is still uncertain, but recent experiments implicate the presence of a cell type in MG thymus which may be involved in autosensitization to AChR.
D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000818:Animals; D000906:Antibodies / Q000032:analysis; D001327:Autoimmune Diseases / Q000209:etiology; D002648:Child; D002675:Child, Preschool; D006801:Humans; D007114:Immunization; D007223:Infant; D007231:Infant, Newborn; D008875:Middle Aged; D008970:Molecular Weight; D009157:Myasthenia Gravis / Q000209:etiology* / Q000503:physiopathology; D009469:Neuromuscular Junction / Q000032:analysis / Q000503:physiopathology; D011950:Receptors, Cholinergic / Q000032:analysis* / Q000276:immunology; D009435:Synaptic Transmission; D013950:Thymus Gland / Q000502:physiology
D000906:Antibodies; D011950:Receptors, Cholinergic