ArticleThe Journal of clinical investigation2004
Specific NEMO mutations impair CD40-mediated c-Rel activation and B cell terminal differentiation.
Article in The Journal of clinical investigation, 2004. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 41 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.
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Who cites it
41 citing papers in PubMed, 86 citations in OpenAlex.
- Partial immune reconstitution of X-linked hyper IgM syndrome with recombinant CD40 ligand.Blood · 2011Trial
- c-Rel plays a key role in deficient activation of B cells from a non-X-linked hyper-IgM patient.Blood · 2006Trial
- NF-κB/RelA signaling required for CD40-induced humoral immunity depends on specific NEMO lysine residues in mice.The EMBO journal · 2026Article
- Inborn errors of human B cell development, differentiation, and function.The Journal of experimental medicine · 2023Review
- B cell receptor signaling in germinal centers prolongs survival and primes B cells for selection.Immunity · 2023Article
- CD40-CD40L in Neurological Disease.International journal of molecular sciences · 2022Review
- miR-182 targeting reprograms tumor-associated macrophages and limits breast cancer progression.Proceedings of the National Academy of Sciences of the United States of America · 2022Article
- Mechanisms of Autoimmune Cell in DA Neuron Apoptosis of Parkinson's Disease: Recent Advancement.Oxidative medicine and cellular longevity · 2022Review
- Update on the Role of NFκB in Promoting Aggressive Phenotypes of Estrogen Receptor-Positive Breast Cancer.Endocrinology · 2020Review
- Regulation of B-cell function by NF-kappaB c-Rel in health and disease.Cellular and molecular life sciences : CMLS · 2020Review
- DNA damage-induced nuclear factor-kappa B activation and its roles in cancer progression.Journal of cancer metastasis and treatment · 2017Article
- Recruitment of A20 by the C-terminal domain of NEMO suppresses NF-κB activation and autoinflammatory disease.Proceedings of the National Academy of Sciences of the United States of America · 2016Article
- The diagnostic approach to monogenic very early onset inflammatory bowel disease.Gastroenterology · 2014Review
- Novel INHAT repressor (NIR) is required for early lymphocyte development.Proceedings of the National Academy of Sciences of the United States of America · 2014Article
- [Humoral immunodeficiency : awareness for better support].The Pan African medical journal · 2014Review
- A novel gain-of-function IKBA mutation underlies ectodermal dysplasia with immunodeficiency and polyendocrinopathy.Journal of clinical immunology · 2013Article
- Immunoglobulin class-switch recombination deficiencies.Arthritis research & therapy · 2012Review
- Default in plasma and intestinal IgA responses during acute infection by simian immunodeficiency virus.Retrovirology · 2012Article
- DNA damage-dependent NF-κB activation: NEMO turns nuclear signaling inside out.Immunological reviews · 2012Review
- Defective nuclear IKKα function in patients with ectodermal dysplasia with immune deficiency.The Journal of clinical investigation · 2012Article
Corrections and comments
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Authors and funding
9 authors at 3 institutions in 1 country.
Funding
Abstract
Hypomorphic mutations in the zinc finger domain of NF-kappaB essential modulator (NEMO) cause X-linked hyper-IgM syndrome with ectodermal dysplasia (XHM-ED). Here we report that patient B cells are characterized by an absence of Ig somatic hypermutation (SHM) and defective class switch recombination (CSR) despite normal induction of activation-induced cytidine deaminase (AID) and Iepsilon-Cepsilon transcripts. This indicates that AID expression alone is insufficient to support neutralizing antibody responses. Furthermore, we show that patient B cells stimulated with CD40 ligand are impaired in both p65 and c-Rel activation, and whereas addition of IL-4 can enhance p65 activity, c-Rel activity remains deficient. This suggests that these NF-kappaB components have different activation requirements and that IL-4 can augment some but not all NEMO-dependent NF-kappaB signaling. Finally, using microarray analysis of patient B cells we identified downstream effects of impaired NF-kappaB activation and candidate factors that may be necessary for CSR and SHM in B cells.
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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.