ArticleBlood2008
Wiskott-Aldrich syndrome protein deficiency in B cells results in impaired peripheral homeostasis.
Article in Blood, 2008. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 49 papers.
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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
49 citing papers in PubMed, 97 citations in OpenAlex.
- Exacerbated experimental arthritis in Wiskott-Aldrich syndrome protein deficiency: modulatory role of regulatory B cells.European journal of immunology · 2014Trial
- WAS Protein Deficiency Disrupts Memory B Cell Formation During Acute LCMV Infection.Journal of clinical immunology · 2026Article
- Immune-related actinopathies at the cross-road of immunodeficiency, autoimmunity and autoinflammation.Nature reviews. Immunology · 2026Review
- WAVE2 Regulates Actin-Dependent Processes Induced by the B Cell Antigen Receptor and Integrins.Cells · 2023Article
- Article
- Not too little, not too much: the impact of mutation types in Wiskott-Aldrich syndrome and RAC2 patients.Clinical and experimental immunology · 2023Review
- Genome editing for primary immunodeficiencies: A therapeutic perspective on Wiskott-Aldrich syndrome.Frontiers in immunology · 2022Review
- ARPC1B binds WASP to control actin polymerization and curtail tonic signaling in B cells.JCI insight · 2021Article
- Autoimmunity in Wiskott-Aldrich Syndrome: Updated Perspectives.The application of clinical genetics · 2021Review
- Targeted gene correction of human hematopoietic stem cells for the treatment of Wiskott - Aldrich Syndrome.Nature communications · 2020Article
- WASP and Mst1 coregulate B-cell development and B-cell receptor signaling.Blood advances · 2020Article
- Higher Incidence of B Cell Malignancies in Primary Immunodeficiencies: A Combination of Intrinsic Genomic Instability and Exocytosis Defects at the Immunological Synapse.Frontiers in immunology · 2020Review
- Congenital Defects in Actin Dynamics of Germinal Center B Cells.Frontiers in immunology · 2019Review
- Autonomous role of Wiskott-Aldrich syndrome platelet deficiency in inducing autoimmunity and inflammation.The Journal of allergy and clinical immunology · 2018Article
- Distinct Roles for Bruton's Tyrosine Kinase in B Cell Immune Synapse Formation.Frontiers in immunology · 2018Article
- Cytoskeletal control of B cell responses to antigens.Nature reviews. Immunology · 2017Review
- Article
- Spontaneous germinal centers and autoimmunity.Autoimmunity · 2017Review
- A Chemoattractant-Guided Walk Through Lymphopoiesis: From Hematopoietic Stem Cells to Mature B Lymphocytes.Advances in immunology · 2017Review
- Article
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Authors and funding
9 authors at 5 institutions in 3 countries.
Funding
Abstract
To more precisely identify the B-cell phenotype in Wiskott-Aldrich syndrome (WAS), we used 3 distinct murine in vivo models to define the cell intrinsic requirements for WAS protein (WASp) in central versus peripheral B-cell development. Whereas WASp is dispensable for early bone marrow B-cell development, WASp deficiency results in a marked reduction in each of the major mature peripheral B-cell subsets, exerting the greatest impact on marginal zone and B1a B cells. Using in vivo bromodeoxyuridine labeling and in vitro functional assays, we show that these deficits reflect altered peripheral homeostasis, partially resulting from an impairment in integrin function, rather than a developmental defect. Consistent with these observations, we also show that: (1) WASp expression levels increase with cell maturity, peaking in those subsets exhibiting the greatest sensitivity to WASp deficiency; (2) WASp(+) murine B cells exhibit a marked selective advantage beginning at the late transitional B-cell stage; and (3) a similar in vivo selective advantage is manifest by mature WASp(+) human B cells. Together, our data provide a better understanding of the clinical phenotype of WAS and suggest that gene therapy might be a useful approach to rescue altered B-cell homeostasis in this disease.
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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.