ArticleThe Journal of neuroscience : the official journal of the Society for Neuroscience2014
Identification and characterization of GABA(A) receptor autoantibodies in autoimmune encephalitis.
Article in The Journal of neuroscience : the official journal of the Society for Neuroscience, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 58 papers, 1 of them a synthesis that pooled it.
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Who cites it
58 citing papers in PubMed, 1 synthesis or guideline pooled it, 125 citations in OpenAlex.
- Anti-gamma-aminobutyric acid receptor type A encephalitis: a review.Current opinion in neurology · 2020Pooled it
- Novel methods for neural IgG characterization and validation in autoimmune neurologic disorders.Journal of immunological methods · 2026Review
- Clinical and neuroradiological evolution of thymoma-associated anti-GABAA receptor autoimmune encephalitis: insights from a clinical case report.Journal of neurology · 2025Article
- Autoantibodies and Inflammation in Schizophrenia.Psychiatry and clinical neurosciences · 2025Review
- Autoimmune encephalitis-associated epilepsy.Nature reviews. Neurology · 2025Review
- Individualized therapy guided by single-cell sequencing in anti-GABATranslational psychiatry · 2025Article
- Antibodies in neurological diseases: Established, emerging, explorative.Immunological reviews · 2024Review
- Unique association of anti-GABANeurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2024Article
- Review
- Coupling of Cortical Hyperintense Signals and Increased Glucose Metabolism in a Case of Anti-GABAInternal medicine (Tokyo, Japan) · 2023Article
- Immune Mechanisms in Epileptogenesis: Update on Diagnosis and Treatment of Autoimmune Epilepsy Syndromes.Drugs · 2023Review
- The JAK-STAT Signaling Pathway in Epilepsy.Current neuropharmacology · 2023Review
- The micro and macro interactions in acute autoimmune encephalitis: a study of resting-state EEG.Frontiers in neurology · 2023Article
- Brain blood vessel autoantibodies in patients with NMDA and GABAFrontiers in cellular neuroscience · 2023Article
- GABA-A Receptor Encephalitis After Autologous Hematopoietic Stem Cell Transplant forMultiple Myeloma: Three Cases and Literature Review.Neurology(R) neuroimmunology & neuroinflammation · 2022Review
- Stiff person syndrome spectrum disorders; more than meets the eye.Journal of neuroimmunology · 2022Review
- Immunity, Ion Channels and Epilepsy.International journal of molecular sciences · 2022Review
- MRI Characteristics of Autoimmune Encephalitis With Autoantibodies to GABAA Receptor: A Case Series.Neurology(R) neuroimmunology & neuroinflammation · 2022Article
- GABAInternational journal of molecular sciences · 2022Article
- Immunotherapy-responsive Non-paraneoplastic Encephalitis with Antibodies against GAD, LGI1, and GABAInternal medicine (Tokyo, Japan) · 2022Article
Corrections and comments
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Authors and funding
10 authors at 5 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Autoimmune forms of encephalitis have been associated with autoantibodies against synaptic cell surface antigens such as NMDA- and AMPA-type glutamate receptors, GABA(B) receptor, and LGI1. However, it remains unclear how many synaptic autoantigens are yet to be defined. Using immunoproteomics, we identified autoantibodies against the GABA(A) receptor in human sera from two patients diagnosed with encephalitis who presented with cognitive impairment and multifocal brain MRI abnormalities. Both patients had antibodies directed against the extracellular epitope of the β3 subunit of the GABA(A) receptor. The β3-subunit-containing GABA(A) receptor was a major target of the patients' serum antibodies in rat hippocampal neurons because the serum reactivity to the neuronal surface was greatly decreased by 80% when the β3 subunit was knocked down. Our developed multiplex ELISA testing showed that both patients had similar levels of GABA(A) receptor antibodies, one patient also had a low level of LGI1 antibodies, and the other also had CASPR2 antibodies. Application of the patients' serum at the time of symptom presentation of encephalitis to rat hippocampal neuron cultures specifically decreased both synaptic and surface GABA(A) receptors. Furthermore, treatment of neurons with the patients' serum selectively reduced miniature IPSC amplitude and frequency without affecting miniature EPSCs. These results strongly suggest that the patients' GABA(A) receptor antibodies play a central role in the patients' symptoms. Therefore, this study establishes anti-GABA(A) receptor encephalitis and expands the pathogenic roles of GABA(A) receptor autoantibodies.
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