ArticleThe Journal of clinical investigation2006
Selective generation of functional somatically mutated IgM+CD27+, but not Ig isotype-switched, memory B cells in X-linked lymphoproliferative disease.
Article in The Journal of clinical investigation, 2006. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 55 papers.
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Who cites it
55 citing papers in PubMed, 133 citations in OpenAlex.
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- Tingible body macrophages arise from lymph node-resident precursors and uptake B cells by dendrites.The Journal of experimental medicine · 2023Article
- The hyperinflammatory spectrum: from defects in cytotoxicity to cytokine control.Frontiers in immunology · 2023Review
- Genome Editing With TALEN, CRISPR-Cas9 and CRISPR-Cas12a in Combination With AAV6 Homology Donor Restores T Cell Function for XLP.Frontiers in genome editing · 2022Article
- Case Report: Novel Splicing Variant inFrontiers in pediatrics · 2022Article
- Intrinsic Defects in B Cell Development and Differentiation, T Cell Exhaustion and Altered Unconventional T Cell Generation Characterize Human Adenosine Deaminase Type 2 Deficiency.Journal of clinical immunology · 2021Article
- SLAM Associated Protein Signaling in T Cells: Tilting the Balance Toward Autoimmunity.Frontiers in immunology · 2021Review
- Rapid identification and characterization of infected cells in blood during chronic active Epstein-Barr virus infection.The Journal of experimental medicine · 2020Article
- Clinical, Immunological, and Functional Characterization of Six Patients with Very High IgM Levels.Journal of clinical medicine · 2020Article
- NK cell recognition of hematopoietic cells by SLAM-SAP families.Cellular & molecular immunology · 2019Review
- Differentiation of Common Variable Immunodeficiency From IgG Deficiency.The journal of allergy and clinical immunology. In practice · 2019Article
- Flow Cytometric-Based Analysis of Defects in Lymphocyte Differentiation and Function Due to Inborn Errors of Immunity.Frontiers in immunology · 2019Review
- X-Linked Lymphoproliferative Disease Type 1: A Clinical and Molecular Perspective.Frontiers in immunology · 2018Review
- T Cells Regulate Peripheral Naive Mature B Cell Survival by Cell-Cell Contact Mediated through SLAMF6 and SAP.Journal of immunology (Baltimore, Md. : 1950) · 2017Article
- Pathogenesis, diagnosis and therapeutic strategies in WHIM syndrome immunodeficiency.Expert opinion on orphan drugs · 2017Article
- Naïve and memory B cells exhibit distinct biochemical responses following BCR engagement.Immunology and cell biology · 2016Article
- Human T Follicular Helper Cells in Primary Immunodeficiency: Quality Just as Important as Quantity.Journal of clinical immunology · 2016Review
- CRISPR-Mediated Slamf1Δ/Δ Slamf5Δ/Δ Slamf6Δ/Δ Triple Gene Disruption Reveals NKT Cell Defects but Not T Follicular Helper Cell Defects.PloS one · 2016Article
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Authors and funding
8 authors at 1 institution in 1 country.
Funding
Abstract
Individuals with X-linked lymphoproliferative disease (XLP) display defects in B cell differentiation in vivo. Specifically, XLP patients do not generate a normal number of CD27 memory B cells, and those few that are present are IgM. Recent studies have suggested that IgMCD27 B cells are not true memory cells, but rather B cells that guard against T cell-independent pathogens. Here we show that human XLP IgMCD27 B cells resemble normal memory B cells both morphologically and phenotypically. Additionally, IgMCD27 B cells exhibited functional characteristics of normal memory B cells, including the ability to secrete more Ig than naive B cells in response to both T cell-dependent and -independent stimuli. Analysis of spleens from XLP patients revealed a paucity of germinal centers (GCs), and the rare GCs detected were poorly formed. Despite this, Ig variable region genes expressed by XLP IgMCD27 B cells had undergone somatic hypermutation to an extent comparable to that of normal memory B cells. These findings reveal a differential requirement for the generation of IgM and Ig isotype-switched memory B cells, with the latter only being generated by fully formed GCs. Production of affinity-matured IgM by IgMCD27 B cells may protect against pathogens to which a normal immune response is elicited in XLP patients.
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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.