ArticleThe Journal of experimental medicine2004
Bruton's tyrosine kinase is essential for human B cell tolerance.
Article in The Journal of experimental medicine, 2004. 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 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.
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Who cites it
86 citing papers in PubMed, 139 citations in OpenAlex.
- Trial
- Defective B cell tolerance in adenosine deaminase deficiency is corrected by gene therapy.The Journal of clinical investigation · 2012Trial
- Unmutated and mutated chronic lymphocytic leukemias derive from self-reactive B cell precursors despite expressing different antibody reactivity.The Journal of clinical investigation · 2005Trial
- Bruton's Tyrosine Kinase Inhibitors: Mechanisms, Efficacy, Toxicities and Applications for the Treatment of Human Diseases.MedComm · 2026Review
- Review
- Failure of early B cell tolerance and possible BCR signaling dysregulation underlie NF155-mediated autoimmune nodopathies.Proceedings of the National Academy of Sciences of the United States of America · 2026Article
- Human genetic influences on early B cell development.Journal of human immunity · 2025Review
- IgA nephropathy in a child with X-linked agammaglobulinemia: a case report.BMC pediatrics · 2024Article
- Inborn errors of human B cell development, differentiation, and function.The Journal of experimental medicine · 2023Review
- Rilzabrutinib, a reversible covalent Bruton's tyrosine kinase inhibitor: Absorption, metabolism, excretion, and absolute bioavailability in healthy participants.Clinical and translational science · 2023Article
- Assessment of autoantibodies in paediatric population with primary immunodeficiencies: a pilot study.BMC immunology · 2023Article
- Bruton tyrosine kinase inhibitors for multiple sclerosis.Nature reviews. Neurology · 2023Review
- [Development of autoimmunity in patients with primary immunodeficiencies].Revista medica del Instituto Mexicano del Seguro Social · 2023Article
- Review
- Diagnostic Challenges in Patients with Inborn Errors of Immunity with Different Manifestations of Immune Dysregulation.Journal of clinical medicine · 2022Review
- Retrospective study of 98 patients with X-linked agammaglobulinemia complicated with arthritis.Clinical rheumatology · 2022Article
- B cell repertoire in patients with a novel BTK mutation: expanding the spectrum of atypical X-linked agammaglobulinemia.Immunologic research · 2022Article
- Effects of B-Cell Lymphoma on the Immune System and Immune Recovery after Treatment: The Paradigm of Targeted Therapy.International journal of molecular sciences · 2022Review
- Positive and negative selection shape the human naive B cell repertoire.The Journal of clinical investigation · 2022Article
- B Cells in Primary Membranous Nephropathy: Escape from Immune Tolerance and Implications for Patient Management.International journal of molecular sciences · 2021Review
26 more citing papers are in PubMed but not listed here.
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
5 authors at 3 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Most polyreactive and antinuclear antibodies are removed from the human antibody repertoire during B cell development. To elucidate how B cell receptor (BCR) signaling may regulate human B cell tolerance, we tested the specificity of recombinant antibodies from single peripheral B cells isolated from patients suffering from X-linked agammaglobulinemia (XLA). These patients carry mutations in the Bruton's tyrosine kinase (BTK) gene that encode an essential BCR signaling component. We find that in the absence of Btk, peripheral B cells show a distinct antibody repertoire consistent with extensive secondary V(D)J recombination. Nevertheless, XLA B cells are enriched in autoreactive clones. Our results demonstrate that Btk is essential in regulating thresholds for human B cell tolerance.
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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.