Cited by 123Myasthenia Gravis and ThymomaNicotinic Acetylcholine Receptors StudyAutoimmune Neurological Disorders and Treatments

Autonomic ganglia, acetylcholine receptor antibodies, and autoimmune ganglionopathy

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Vernino, Steven, Hopkins, Steve, Wang, Zhengbei·2009·Autonomic Neuroscience

Abstract

Nicotinic acetylcholine receptors (AChR) are ligand-gated cation channels that are present throughout the nervous system. The ganglionic (alpha3-type) neuronal AChR mediates fast synaptic transmission in sympathetic, parasympathetic and enteric autonomic ganglia. Autonomic ganglia are an important site of neural integration and regulation of autonomic reflexes. Impaired cholinergic ganglionic synaptic transmission is one important cause of autonomic failure. Ganglionic AChR antibodies are found in many patients with autoimmune autonomic ganglionopathy (AAG). These antibodies recognize the alpha3 subunit of the ganglionic AChR, and thus do not bind non-specifically to other nicotinic AChR. Patients with high levels of ganglionic AChR antibodies typically present with rapid onset of severe autonomic failure, with orthostatic hypotension, gastrointestinal dysmotility, anhidrosis, bladder dysfunction and sicca symptoms. Impaired pupillary light reflex is often seen. Like myasthenia gravis, AAG is an antibody-mediated neurological disorder. Antibodies from patients with AAG inhibit ganglionic AChR currents and impair transmission in autonomic ganglia. An animal model of AAG in the rabbit recapitulates the important clinical features of the human disease and provides additional evidence that AAG is an antibody-mediated disorder caused by impairment of synaptic transmission in autonomic ganglia.

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Crossref · 10.1016/j.autneu.2008.09.005

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  • Ganglionic acetylcholine receptor antibodies are implicated in autoimmune autonomic ganglionopathy, an acute pandysautonomia with characteristically severe gastrointestinal involvement.

    “Ganglionic acetylcholine receptor (G-AChR) antibodies are implicated in autoimmune autonomic ganglionopathy, an acute pandysautonomia with characteristically severe gastrointestinal involvement.”

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  1. Myers AC 2000 Anatomical characteristics of tonic and phasic postganglionic neurons in guinea pig bronchial parasympathetic ganglia J Comp Neurol 419 439 450 10742714

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Authors
Vernino, S., Hopkins, S., & Wang, Z.
Affiliations
The University of Texas Southwestern Medical Center (US)
Corresponding author
Vernino, Steven
Venue
Autonomic Neuroscience
Publisher
Elsevier BV
Published
2009-03
Volume
146
Issue
1-2
Pages
3-7
ISSN
1566-0702 (ISSN-L)
OpenAlex
W2108751406
Cited by
123
References
21
Citations by year
2026: 11 · 2025: 2 · 2024: 8 · 2023: 3 · 2022: 6 · 2021: 10 · 2020: 9 · 2019: 3 · 2018: 7 · 2017: 5 · 2016: 10 · 2015: 6 · 2014: 7 · 2013: 12 · 2012: 5
FWCI
4.06
Type
journal article
Language
en
Open access
Locations
  • Autonomic Neuroscience (publishedVersion)
  • PubMed (publishedVersion)
  • PubMed Central (acceptedVersion)
Funders
National Institute of Neurological Disorders and Stroke (P50NS 32352, P50NS032352, R01NS48077, R01NS048077)
Topics
  • Health Sciences › Medicine › Neurology › Myasthenia Gravis and Thymoma
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Nicotinic Acetylcholine Receptors Study
  • Health Sciences › Medicine › Neurology › Autoimmune Neurological Disorders and Treatments
Keywords
alpha3 subunit, autonomic ganglia, autonomic failure, cholinergic neurotransmission, orthostatic hypotension, gastrointestinal motility disorders, anhidrosis, bladder dysfunction, sicca symptoms, pupillary light reflex, rabbit model, antibody-mediated autoimmunity
MeSH terms
Adult, Aged, Aged, 80 and over, Animals, Autoantibodies, Autoantigens, Female, Ganglia, Autonomic, Humans, Immunization, Passive, Male, Middle Aged, Myasthenia Gravis, Synaptic Transmission, Rabbits, Receptors, Nicotinic, Sensitivity and Specificity, Autoimmune Diseases of the Nervous System, Paraneoplastic Syndromes, Nervous System, Nervous System Autoimmune Disease, Experimental, Primary Dysautonomias, Young Adult

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