ArticleThe Journal of clinical investigation2013
CVID-associated TACI mutations affect autoreactive B cell selection and activation.
Article in The Journal of clinical investigation, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 86 papers.
What it found
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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.
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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.
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Who cites it
86 citing papers in PubMed, 158 citations in OpenAlex.
- Common variable immunodeficiency-associated endotoxemia promotes early commitment to the T follicular lineage.The Journal of allergy and clinical immunology · 2019Trial
- Non-parametric Heat Map Representation of Flow Cytometry Data: Identifying Cellular Changes Associated With Genetic Immunodeficiency Disorders.Frontiers in immunology · 2019Trial
- Patients with common variable immunodeficiency with autoimmune cytopenias exhibit hyperplastic yet inefficient germinal center responses.The Journal of allergy and clinical immunology · 2019Trial
- The immunoregulator soluble TACI is released by ADAM10 and reflects B cell activation in autoimmunity.Journal of immunology (Baltimore, Md. : 1950) · 2015Trial
- BAFF and APRIL Receptors in B Cell Immunity and Autoimmunity.Immunological reviews · 2026Review
- Coexistent alterations of BAFF and B-cell phenotypes in complicated CVID course.The Journal of allergy and clinical immunology · 2026Article
- Navigating primary and secondary immunodeficiency intersections: how to find IEI hidden within SID.Allergy, asthma, and clinical immunology : official journal of the Canadian Society of Allergy and Clinical Immunology · 2026Review
- IgA defects in CVID lead to bacterial translocation, increased serum γ-interferon, and BAFF.Journal of human immunity · 2026Article
- TNFRSF13B Variant-Induced TACI Dysregulation Underlies CAEBV Pathogenesis.Journal of clinical immunology · 2026Article
- Re-evaluation of the contribution ofJournal of human immunity · 2025Article
- Human genetic influences on early B cell development.Journal of human immunity · 2025Review
- Pathogenic TNFRSF13B Variant in an Adult Japanese Patient with Common Variable Immunodeficiency.Internal medicine (Tokyo, Japan) · 2025Article
- Common variable immunodeficiency and autoimmune diseases: A 10-year single-center experience.Archives of rheumatology · 2024Article
- The common variable immunodeficiency IgM repertoire narrowly recognizes erythrocyte and platelet glycans.The Journal of allergy and clinical immunology · 2024Article
- Analysis of rare genetic variants inFrontiers in genetics · 2024Article
- Inborn Errors of Immunity and Cytokine Storm Syndromes.Advances in experimental medicine and biology · 2024Review
- TLR9 ligand sequestration by chemokine CXCL4 negatively affects central B cell tolerance.The Journal of experimental medicine · 2023Article
- Article
- BAFF system expression in double negative 2, activated naïve and activated memory B cells in systemic lupus erythematosus.Frontiers in immunology · 2023Article
- Lymphoid stromal cells - potential implications for the pathogenesis of CVID.Frontiers in immunology · 2023Review
26 more citing papers are in PubMed but not listed here.
Corrections and comments
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Authors and funding
18 authors at 7 institutions in 4 countries.
Funding
Abstract
Common variable immune deficiency (CVID) is an assorted group of primary diseases that clinically manifest with antibody deficiency, infection susceptibility, and autoimmunity. Heterozygous mutations in the gene encoding the tumor necrosis factor receptor superfamily member TACI are associated with CVID and autoimmune manifestations, whereas two mutated alleles prevent autoimmunity. To assess how the number of TACI mutations affects B cell activation and tolerance checkpoints, we analyzed healthy individuals and CVID patients carrying one or two TACI mutations. We found that TACI interacts with the cleaved, mature forms of TLR7 and TLR9 and plays an important role during B cell activation and the central removal of autoreactive B cells in healthy donors and CVID patients. However, only subjects with a single TACI mutation displayed a breached immune tolerance and secreted antinuclear antibodies (ANAs). These antibodies were associated with the presence of circulating B cell lymphoma 6-expressing T follicular helper (Tfh) cells, likely stimulating autoreactive B cells. Thus, TACI mutations may favor CVID by altering B cell activation with coincident impairment of central B cell tolerance, whereas residual B cell responsiveness in patients with one, but not two, TACI mutations enables autoimmune complications.
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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.