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Autonomic ganglia, acetylcholine receptor antibodies, and autoimmune ganglionopathy
Open accessAbstract
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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“Ganglionic acetylcholine receptor (G-AChR) antibodies are implicated in autoimmune autonomic ganglionopathy, an acute pandysautonomia with characteristically severe gastrointestinal involvement.”
From Autonomic nervous system and gastrointestinal manifestations with an emphasis on gastric dysfunction (2026) · cites this paper as Vernino et al., 2009
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Autoimmune Autonomic Ganglionopathy
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Self-citation
Metadata
- Authors
- Vernino, S., Hopkins, S., & Wang, Z.
- Affiliations
- The University of Texas Southwestern Medical Center (US)
- Corresponding author
- Vernino, Steven
- ORCID
- Vernino, Steven
- Venue
- Autonomic Neuroscience
- Publisher
- Elsevier BV
- Published
- 2009-03
- Volume
- 146
- Issue
- 1-2
- Pages
- 3-7
- ISSN
- 1566-0702 (ISSN-L)
- PMC
- PMC2677210
- 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
- green
- www.ncbi.nlm.nih.gov
- 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 › Clinical Neurology › Myasthenia Gravis and Thymoma
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Nicotinic Acetylcholine Receptors Study
- Health Sciences › Medicine › Clinical 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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