Neuronal acetylcholine receptor autoimmunity

Research output: Chapter in Book/Report/Conference proceedingConference contribution

13 Scopus citations


Nicotinic acetylcholine receptors (AChRs) are ligand-gated cation channels that are present throughout the nervous system. The muscle AChR mediates transmission at the neuromuscular junction; antibodies against the muscle AChR are the cause of myasthenia gravis. There are numerous subtypes of AChRs present throughout the nervous system. These neuronal AChRs are closely related to the muscle AChR structurally, but are pharmacologically and immunologically distinct. The ganglionic (α3-type) neuronal AChR mediates fast synaptic transmission in sympathetic, parasympathetic, and enteric autonomic ganglia. Ganglionic AChR antibodies are found in up to 50% of patients with autoimmune autonomic ganglionopathy (AAG). Patients with AAG present with severe autonomic failure. Major clinical features include orthostatic hypotension, gastrointestinal dysmotility, anhidrosis, bladder dysfunction, and sicca symptoms. Impaired pupillary light reflex is often seen. Like myasthenia, AAG is an antibody-mediated neurological disorder. Patients may improve with plasma exchange treatment or other immunomodulatory treatment. Experimental AAG, which recapitulates many of the features of the human disease, can be induced in rabbits by immunization against the ganglionic AChR or by passive transfer of antibodies to mice. Ganglionic AChR IgG inhibits nicotinic membrane current in cultured neuroblastoma cells. Central nervous system neuronal AChRs may also be targets of autoimmunity. For example, antibodies against α7-type AChRs have been identified in a few patients with encephalitis. It is still unclear if antibodies against receptors in the central nervous system can directly cause disease.

Original languageEnglish (US)
Title of host publicationMyasthenia Gravis and Related Disorders 11th International Conference
PublisherBlackwell Publishing Inc.
Number of pages5
ISBN (Print)9781573316873
StatePublished - Jun 2008

Publication series

NameAnnals of the New York Academy of Sciences
ISSN (Print)0077-8923
ISSN (Electronic)1749-6632


  • Autonomic neuropathy
  • Gastrointestinal dysmotility
  • Orthostatic hypotension
  • Thymoma

ASJC Scopus subject areas

  • Neuroscience(all)
  • Biochemistry, Genetics and Molecular Biology(all)
  • History and Philosophy of Science


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