Abstract
In myasthenia gravis (MG), autoantibodies bind to the alpha1 subunit and other subunits of the muscle nicotinic acetylcholine receptor (AChR). Autoimmune autonomic ganglionopathy (AAG) is an antibody-mediated neurological disorder caused by antibodies against neuronal AChRs in autonomic ganglia. Subunits of muscle and neuronal AChR are homologous. We examined the specificity of AChR antibodies in patients with MG and AAG. Ganglionic AChR autoantibodies found in AAG patients are specific for AChRs containing the alpha3 subunit. Muscle and ganglionic AChR antibody specificities are distinct. Antibody crossreactivity between AChRs with different alpha subunits is uncommon but can occur.
Publication types
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Comparative Study
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Research Support, N.I.H., Extramural
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Research Support, Non-U.S. Gov't
MeSH terms
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Animals
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Autoantibodies / biosynthesis
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Autoantibodies / chemistry*
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Autoantibodies / metabolism
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Autoimmune Diseases of the Nervous System / immunology
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Autoimmune Diseases of the Nervous System / metabolism
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Autonomic Nervous System Diseases / immunology
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Autonomic Nervous System Diseases / metabolism
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Cell Line
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Cell Line, Tumor
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Ganglia, Autonomic / immunology*
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Ganglia, Autonomic / metabolism
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Humans
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Mice
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Mice, Nude
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Muscle, Skeletal / immunology*
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Muscle, Skeletal / metabolism
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Rabbits
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Receptors, Muscarinic / immunology*
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Receptors, Muscarinic / metabolism
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Receptors, Nicotinic / immunology*
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Receptors, Nicotinic / metabolism
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alpha7 Nicotinic Acetylcholine Receptor
Substances
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Autoantibodies
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Chrna7 protein, human
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Chrna7 protein, mouse
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Receptors, Muscarinic
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Receptors, Nicotinic
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alpha7 Nicotinic Acetylcholine Receptor
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nicotinic receptor alpha3beta2
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nicotinic receptor alpha3beta4
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nicotinic receptor alpha4beta2