The DOI matches its Crossref record; 24 of the 24 references it cites were found in a registry or an index.
Wiskott–Aldrich syndrome protein is required for NK cell cytotoxicity and colocalizes with actin to NK cell-activating immunologic synapses
Open accessAbstract
The Wiskott–Aldrich syndrome (WAS) is a primary immunodeficiency disorder caused by a mutation in WAS protein (WASp) that results in defective actin polymerization. Although the function of many hematopoietic cells requires WASp, the specific expression and function of this molecule in natural killer (NK) cells is unknown. Here, we report that WAS patients have increased percentages of peripheral blood NK cells and that fresh enriched NK cells from two patients with a WASp mutation have defective cytolytic function. In normal NK cells, WASp was expressed and localized to the activating immunologic synapse (IS) with filamentous actin (F-actin). Perforin also localized to the NK cell-activating IS but at a lesser frequency than F-actin and WASp. The accumulation of F-actin and WASp at the activating IS was decreased significantly in NK cells that had been treated with the inhibitor of actin polymerization, cytochalasin D. NK cells from WAS patients lacked expression of WASp and accumulated F-actin at the activating IS infrequently. Thus, WASp has an important function in NK cells. In patients with WASp mutations, the resulting NK cell defects are likely to contribute to their disease.
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Cites
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Self-citationWIP regulates N-WASP-mediated actin polymerization and filopodium formation
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Cited by
Perforin and granzymes: function, dysfunction and human pathology
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Lentiviral Hematopoietic Stem Cell Gene Therapy in Patients with Wiskott-Aldrich Syndrome
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Self-citationGene therapy for Wiskott-Aldrich syndrome–long-term efficacy and genotoxicity
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Stem-Cell Gene Therapy for the Wiskott–Aldrich Syndrome
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Self-citationWASP: a key immunological multitasker
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Metadata
- Authors
- Orange, J. S., Ramesh, N., Remold-O'Donnell, E., Sasahara, Y., Koopman, L., Byrne, M., Bonilla, F. A., Rosen, F. S., Geha, R. S., & Strominger, J. L.
- Venue
- Proceedings of the National Academy of Sciences
- Published
- 2002-08-12
- Volume
- 99
- Issue
- 17
- Pages
- 11351-11356
- ISSN
- 0027-8424 (ISSN-L)
- 1091-6490
- OpenAlex
- W2053784823
- Cited by
- 295
- References
- 24
- FWCI
- 4.10
- Type
- journal article
- Language
- en
- Open access
- green
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- CoChat. (2026). Citation check: Wiskott–Aldrich syndrome protein is required for NK cell cytotoxicity and colocalizes with actin to NK cell-activating immunologic synapses. https://app.cochat.ai/papers/10.1073/pnas.162376099
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- Orange, J. S., Ramesh, N., Remold-O'Donnell, E., Sasahara, Y., Koopman, L., Byrne, M., Bonilla, F. A., Rosen, F. S., Geha, R. S., & Strominger, J. L. (2002). Wiskott–Aldrich syndrome protein is required for NK cell cytotoxicity and colocalizes with actin to NK cell-activating immunologic synapses. Proceedings of the National Academy of Sciences. https://doi.org/10.1073/pnas.162376099
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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
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