ArticleBrain : a journal of neurology2024
HLA-DQB1*05 subtypes and not DRB1*10:01 mediates risk in anti-IgLON5 disease.
Article in Brain : a journal of neurology, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 26 papers, 1 of them a synthesis that pooled it.
What it found
Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.
The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
26 citing papers in PubMed, 1 synthesis or guideline pooled it, 31 citations in OpenAlex.
- Anti-IgLON5 Disease: A Systematic Review and Meta-Analysis.European journal of neurology · 2025Pooled it
- The cascade to pathogenicity in autoantibody-mediated CNS diseases.Brain : a journal of neurology · 2026Review
- Neuronal Antibodies in Patients With Atypical Parkinsonism.Neurology(R) neuroimmunology & neuroinflammation · 2026Observational
- Prognostic Determinants of Presentation and Outcome in Anti-IgLON5 Disease.Neurology(R) neuroimmunology & neuroinflammation · 2026Observational
- Article
- Human IgLON5 Antibodies Reduce Neuronal IgLON5 Clusters and Cause Motor Dysfunction in Mice.Neurology(R) neuroimmunology & neuroinflammation · 2026Article
- Clinical features, outcome and HLA subtypes in Eastern patients with anti-IgLON5 disease: a multicenter study.Orphanet journal of rare diseases · 2026Article
- [Role of HLA genetic variants in autoimmune diseases].Zhong nan da xue xue bao. Yi xue ban = Journal of Central South University. Medical sciences · 2026Review
- Brainstem pathology in anti-IgLON5 disease: new insights into early events and tau progression.Brain : a journal of neurology · 2026Article
- HLA Class I Haplotype in Glutamic Acid Decarboxylase Antibody-Associated Neurological Disease.European journal of neurology · 2026Article
- Brain atrophy patterns in anti-IgLON5 disease.Brain : a journal of neurology · 2026Article
- Clinical utility of human leukocyte antigen genotyping and immunoglobulin G4 autoantibody testing in autoimmune neurological diseases: A focused minireview.World journal of biological chemistry · 2026Review
- A patient with anti-IgLON5 disease associated with cerebral hemorrhage, multiple ischemic strokes, and kidney failure: case report.Journal of neurology · 2026Article
- Distinct neuroinflammatory profiles in progressive supranuclear palsy associated withBrain communications · 2026Article
- Autoimmune encephalitis.Nature reviews. Disease primers · 2025Review
- Causes of Death in Anti-IgLON5 Disease: A Novel Case Report and Systematic Literature Review.Brain sciences · 2025Article
- Neuroinflammation distinguishes HLA haplotypes in progressive supranuclear palsy.medRxiv : the preprint server for health sciences · 2025Article
- Neurodegeneration and the immune system: lessons from autoimmune encephalitis.Journal of neurology · 2025Review
- Application of the anti-IgLON5 disease composite score to assess severity, clinical course, and mortality in a French cohort.Journal of neurology · 2025Article
- Patterns of Tau pathology in patients with anti-IgLON5 disease visualized by Florzolotau (18F) PET.Journal of neurology · 2025Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
42 authors at 20 institutions in 10 countries.
Funding
Abstract
Anti-IgLON5 disease is a rare and likely underdiagnosed subtype of autoimmune encephalitis. The disease displays a heterogeneous phenotype that includes sleep, movement and bulbar-associated dysfunction. The presence of IgLON5-antibodies in CSF/serum, together with a strong association with HLA-DRB1*10:01∼DQB1*05:01, supports an autoimmune basis. In this study, a multicentric human leukocyte antigen (HLA) study of 87 anti-IgLON5 patients revealed a stronger association with HLA-DQ than HLA-DR. Specifically, we identified a predisposing rank-wise association with HLA-DQA1*01:05∼DQB1*05:01, HLA-DQA1*01:01∼DQB1*05:01 and HLA-DQA1*01:04∼DQB1*05:03 in 85% of patients. HLA sequences and binding cores for these three DQ heterodimers were similar, unlike those of linked DRB1 alleles, supporting a causal link to HLA-DQ. This association was further reflected in an increasingly later age of onset across each genotype group, with a delay of up to 11 years, while HLA-DQ-dosage dependent effects were also suggested by reduced risk in the presence of non-predisposing DQ1 alleles. The functional relevance of the observed HLA-DQ molecules was studied with competition binding assays. These proof-of-concept experiments revealed preferential binding of IgLON5 in a post-translationally modified, but not native, state to all three risk-associated HLA-DQ receptors. Further, a deamidated peptide from the Ig2-domain of IgLON5 activated T cells in two patients, compared with one control carrying HLA-DQA1*01:05∼DQB1*05:01. Taken together, these data support a HLA-DQ-mediated T-cell response to IgLON5 as a potentially key step in the initiation of autoimmunity in this disease.
Indexed as
Identifiers
What OpenQuestion holds
Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.