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dc.contributor.author | Lee, Pamela P | |
dc.contributor.author | Lobato-Marquez, Damian | |
dc.contributor.author | Pramanik, Nayani | |
dc.contributor.author | Sirianni, Andrea | |
dc.contributor.author | Daza-Cajigal, Vanessa | |
dc.contributor.author | Rivers, Elizabeth | |
dc.contributor.author | Cavazza, Alessia | |
dc.contributor.author | Bouma, Gerben | |
dc.contributor.author | Moulding, Dale | |
dc.contributor.author | Hultenby, Kjell | |
dc.contributor.author | Westerberg, Lisa S | |
dc.contributor.author | Hollinshead, Michael | |
dc.contributor.author | Lau, Yu-Lung | |
dc.contributor.author | Burns, Siobhan O | |
dc.contributor.author | Mostowy, Serge | |
dc.contributor.author | Bajaj-Elliott, Mona | |
dc.contributor.author | Thrasher, Adrian J | |
dc.date.accessioned | 2024-07-11T09:10:47Z | |
dc.date.available | 2024-07-11T09:10:47Z | |
dc.date.issued | 2017-11-17 | |
dc.identifier.citation | Lee Pamela P, Lobato-Marquez D, Pramanik N, Sirianni A, Daza Cajigal VC, Rivers E, et al. Wiskott-Aldrich syndrome protein regulates autophagy and inflammasome activity in innate immune cells. Nat Commun. 2017 Nov 17;8:1576. | en |
dc.identifier.issn | 2041-1723 | |
dc.identifier.other | http://hdl.handle.net/20.500.13003/9556 | |
dc.identifier.uri | http://hdl.handle.net/20.500.12105/20463 | |
dc.description.abstract | Dysregulation of autophagy and inflammasome activity contributes to the development of auto-inflammatory diseases. Emerging evidence highlights the importance of the actin cytoskeleton in modulating inflammatory responses. Here we show that deficiency of Wiskott-Aldrich syndrome protein (WASp), which signals to the actin cytoskeleton, modulates autophagy and inflammasome function. In a model of sterile inflammation utilizing TLR4 ligation followed by ATP or nigericin treatment, inflammasome activation is enhanced in monocytes from WAS patients and in WAS-knockout mouse dendritic cells. In ex vivo models of enteropathogenic Escherichia coli and Shigella flexneri infection, WASp deficiency causes defective bacterial clearance, excessive inflammasome activation and host cell death that are associated with dysregulated septin cage-like formation, impaired autophagic p62/LC3 recruitment and defective formation of canonical autophagosomes. Taken together, we propose that dysregulation of autophagy and inflammasome activities contribute to the autoinflammatory manifestations of WAS, thereby identifying potential targets for therapeutic intervention. | en |
dc.description.sponsorship | We thank the patients and their parents for their support. This work was supported by grants from the Wellcome Trust (A.J.T., G.B., D.M., E.R., A.C.), Great Ormond Street Hospital Children's Charity (A.J.T., P.P.L.), Wellcome Trust and Lister Institute (S.M.). A.J.T. is supported by both the Wellcome Trust (104807/Z/14/Z) and by the National Institute for Health Research Biomedical Research Centre at Great Ormond Street Hospital for Children NHS Foundation Trust and University College London. | es_ES |
dc.language.iso | eng | en |
dc.publisher | Nature Publishing Group | en |
dc.rights.uri | http://creativecommons.org/licenses/by/4.0/ | * |
dc.subject.mesh | Wiskott-Aldrich Syndrome Protein | * |
dc.subject.mesh | Enteropathogenic Escherichia coli | * |
dc.subject.mesh | Dendritic Cells | * |
dc.subject.mesh | Bacterial Load | * |
dc.subject.mesh | Humans | * |
dc.subject.mesh | THP-1 Cells | * |
dc.subject.mesh | Cell Line, Tumor | * |
dc.subject.mesh | Mice, Inbred C57BL | * |
dc.subject.mesh | Actin Cytoskeleton | * |
dc.subject.mesh | Autophagy | * |
dc.subject.mesh | Immunity, Innate | * |
dc.subject.mesh | Wiskott-Aldrich Syndrome | * |
dc.subject.mesh | NLR Family, Pyrin Domain-Containing 3 Protein | * |
dc.subject.mesh | Monocytes | * |
dc.subject.mesh | Nigericin | * |
dc.subject.mesh | Toll-Like Receptor 4 | * |
dc.subject.mesh | Septins | * |
dc.subject.mesh | Interferon Type I | * |
dc.subject.mesh | Animals | * |
dc.subject.mesh | Inflammasomes | * |
dc.subject.mesh | Mice, Knockout | * |
dc.subject.mesh | Shigella flexneri | * |
dc.subject.mesh | Mice | * |
dc.title | Wiskott-Aldrich syndrome protein regulates autophagy and inflammasome activity in innate immune cells | en |
dc.type | research article | en |
dc.rights.license | Attribution 4.0 International | * |
dc.identifier.pubmedID | 29146903 | es_ES |
dc.format.volume | 8 | es_ES |
dc.format.page | 1576 | es_ES |
dc.identifier.doi | 10.1038/s41467-017-01676-0 | |
dc.relation.publisherversion | https://dx.doi.org/10.1038/s41467-017-01676-0 | en |
dc.identifier.journal | Nature Communications | es_ES |
dc.rights.accessRights | open access | en |
dc.subject.decs | Animales | * |
dc.subject.decs | Línea Celular Tumoral | * |
dc.subject.decs | Ratones Noqueados | * |
dc.subject.decs | Carga Bacteriana | * |
dc.subject.decs | Síndrome de Wiskott-Aldrich | * |
dc.subject.decs | Inmunidad Innata | * |
dc.subject.decs | Interferón Tipo I | * |
dc.subject.decs | Monocitos | * |
dc.subject.decs | Nigericina | * |
dc.subject.decs | Inflamasomas | * |
dc.subject.decs | Receptor Toll-Like 4 | * |
dc.subject.decs | Citoesqueleto de Actina | * |
dc.subject.decs | Autofagia | * |
dc.subject.decs | Humanos | * |
dc.subject.decs | Escherichia coli Enteropat�gena | * |
dc.subject.decs | Ratones Endog�micos C57BL | * |
dc.subject.decs | Septinas | * |
dc.subject.decs | Proteína del Síndrome de Wiskott-Aldrich | * |
dc.subject.decs | Shigella flexneri | * |
dc.subject.decs | Células Dendr�ticas | * |
dc.subject.decs | Ratones | * |
dc.subject.decs | Proteína con Dominio Pirina 3 de la Familia NLR | * |
dc.subject.decs | Células THP-1 | * |
dc.identifier.scopus | 2-s2.0-85034614435 | |
dc.identifier.wos | 415648900008 | |
dc.identifier.pui | L619287601 |
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