ArticleThe Journal of experimental medicine2009
The Wiskott-Aldrich syndrome protein is required for iNKT cell maturation and function.
Article in The Journal of experimental medicine, 2009. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 27 papers.
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Who cites it
27 citing papers in PubMed, 55 citations in OpenAlex.
- How I treat Wiskott-Aldrich syndrome.Blood · 2025Review
- Thymic development of human natural killer T cells: recent advances and implications for immunotherapy.Frontiers in immunology · 2024Review
- Role of NKT Cells during Viral Infection and the Development of NKT Cell-Based Nanovaccines.Vaccines · 2021Review
- Autoimmunity in Wiskott-Aldrich Syndrome: Updated Perspectives.The application of clinical genetics · 2021Review
- Article
- Wiskott-Aldrich syndrome protein: Emerging mechanisms in immunity.European journal of immunology · 2017Review
- Are fat NKT cells born that way?Cellular & molecular immunology · 2017Article
- CD1d Expression and Invariant NKT Cell Responses in Herpesvirus Infections.Frontiers in immunology · 2015Review
- Signal Integration during T Lymphocyte Activation and Function: Lessons from the Wiskott-Aldrich Syndrome.Frontiers in immunology · 2015Review
- Actin cytoskeletal defects in immunodeficiency.Immunological reviews · 2013Review
- Article
- Reduced type I interferon production by dendritic cells and weakened antiviral immunity in patients with Wiskott-Aldrich syndrome protein deficiency.The Journal of allergy and clinical immunology · 2013Article
- Wiskott-Aldrich syndrome protein-mediated actin dynamics control type-I interferon production in plasmacytoid dendritic cells.The Journal of experimental medicine · 2013Article
- Human iNKT and MAIT cells exhibit a PLZF-dependent proapoptotic propensity that is counterbalanced by XIAP.Blood · 2013Article
- Dendritic cell functional improvement in a preclinical model of lentiviral-mediated gene therapy for Wiskott-Aldrich syndrome.Gene therapy · 2012Article
- Wiskott-Aldrich syndrome protein deficiency in innate immune cells leads to mucosal immune dysregulation and colitis in mice.Gastroenterology · 2012Article
- Article
- Autoimmunity in wiskott-Aldrich syndrome: an unsolved enigma.Frontiers in immunology · 2012Article
- Molecular pathogenesis of EBV susceptibility in XLP as revealed by analysis of female carriers with heterozygous expression of SAP.PLoS biology · 2011Article
- Recent insights into the pathobiology of innate immune deficiencies.Current allergy and asthma reports · 2011Review
Corrections and comments
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Authors and funding
17 authors at 9 institutions in 7 countries.
Funding
Abstract
The Wiskott-Aldrich syndrome (WAS) protein (WASp) is a regulator of actin cytoskeleton in hematopoietic cells. Mutations of the WASp gene cause WAS. Although WASp is involved in various immune cell functions, its role in invariant natural killer T (iNKT) cells has never been investigated. Defects of iNKT cells could indeed contribute to several WAS features, such as recurrent infections and high tumor incidence. We found a profound reduction of circulating iNKT cells in WAS patients, directly correlating with the severity of clinical phenotype. To better characterize iNKT cell defect in the absence of WASp, we analyzed was(-/-) mice. iNKT cell numbers were significantly reduced in the thymus and periphery of was(-/-) mice as compared with wild-type controls. Moreover analysis of was(-/-) iNKT cell maturation revealed a complete arrest at the CD44(+) NK1.1(-) intermediate stage. Notably, generation of BM chimeras demonstrated a was(-/-) iNKT cell-autonomous developmental defect. was(-/-) iNKT cells were also functionally impaired, as suggested by the reduced secretion of interleukin 4 and interferon gamma upon in vivo activation. Altogether, these results demonstrate the relevance of WASp in integrating signals critical for development and functional differentiation of iNKT cells and suggest that defects in these cells may play a role in WAS pathology.
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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.