ArticleThe Journal of clinical investigation1999
Defects of T-cell effector function and post-thymic maturation in X-linked hyper-IgM syndrome.
Article in The Journal of clinical investigation, 1999. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT00266513 (Studies of Disorders in Antibody Production and Related Primary Immunodeficiency States), which is not on this map. Cited by 36 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.
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.
Studies of Disorders in Antibody Production and Related Primary Immunodeficiency States
Who cites it
36 citing papers in PubMed, 114 citations in OpenAlex.
- CD40 agonist antibody mediated improvement of chronic Cryptosporidium infection in patients with X-linked hyper IgM syndrome.Clinical immunology (Orlando, Fla.) · 2012Trial
- Partial immune reconstitution of X-linked hyper IgM syndrome with recombinant CD40 ligand.Blood · 2011Trial
- Mechanotransduction governs CD40 function and underlies X-linked hyper-IgM syndrome.Science advances · 2024Article
- Scalable GMP-compliant gene correction of CD4+ T cells with IDLV template functionally validatedMolecular therapy. Methods & clinical development · 2023Article
- Dendritic cells in inborn errors of immunity.Frontiers in immunology · 2023Review
- CD40 Ligand Deficiency in Latin America: Clinical, Immunological, and Genetic Characteristics.Journal of clinical immunology · 2022Article
- Infectious Complications Predict Premature CD8Journal of clinical immunology · 2021Article
- Modeling, optimization, and comparable efficacy of T cell and hematopoietic stem cell gene editing for treating hyper-IgM syndrome.EMBO molecular medicine · 2021Article
- CD40 ligand deficiency causes functional defects of peripheral neutrophils that are improved by exogenous IFN-γ.The Journal of allergy and clinical immunology · 2018Article
- Disseminated Cutaneous Warts in X-Linked Hyper IgM Syndrome.Journal of clinical immunology · 2018Review
- Long-term outcomes of 176 patients with X-linked hyper-IgM syndrome treated with or without hematopoietic cell transplantation.The Journal of allergy and clinical immunology · 2017Observational
- Adaptive Immunity toJournal of fungi (Basel, Switzerland) · 2017Article
- X-linked Hyper IgM Syndrome Presenting as Pulmonary Alveolar Proteinosis.Journal of clinical immunology · 2016Article
- Hyper IgM Syndrome: a Report from the USIDNET Registry.Journal of clinical immunology · 2016Article
- Flow Cytometry, a Versatile Tool for Diagnosis and Monitoring of Primary Immunodeficiencies.Clinical and vaccine immunology : CVI · 2016Review
- ICON: the early diagnosis of congenital immunodeficiencies.Journal of clinical immunology · 2014Review
- The hyper IgM syndromes.Clinical reviews in allergy & immunology · 2014Review
- Recent advances in transplantation for primary immune deficiency diseases: a comprehensive review.Clinical reviews in allergy & immunology · 2014Review
- First report of the Hyper-IgM syndrome Registry of the Latin American Society for Immunodeficiencies: novel mutations, unique infections, and outcomes.Journal of clinical immunology · 2014Article
- Clinical features and genetic analysis of 20 Chinese patients with X-linked hyper-IgM syndrome.Journal of immunology research · 2014Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
6 authors at 5 institutions in 1 country.
Funding
Abstract
X-linked hyper-IgM syndrome (XHIM) results from mutations in the gene encoding for CD40 ligand (CD154). Patients with the syndrome suffer from infections with opportunistic pathogens such as Cryptosporidium and Pneumocystis carinii. In this study, we demonstrate that activated T cells from patients with XHIM produce markedly reduced levels of IFN-gamma, fail to induce antigen-presenting cells to synthesize IL-12, and induce greatly reduced levels of TNF-alpha. In addition, we show that the patients' circulating T lymphocytes of both the CD4(+) and CD8(+) subsets contain a markedly reduced antigen-primed population, as determined by CD45RO expression. Finally, we demonstrate that the defects in antigen priming are likely due to the lack of CD154 expression and insufficient costimulation of T cells by CD80/CD86 interactions. Taken together, this study offers a basis for the increased susceptibility of patients with XHIM to certain opportunistic infections.
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Identifiers
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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.