Acquired Somatic Mutations In Pnh Reveal Long-term Maintenance Of Adaptive Nk Cells Independent Of Hspcs

dc.creatorCorat
dc.creatorMarcus A. F.; Schlums
dc.creatorHeinrich; Wu
dc.creatorChuanfeng; Theorell
dc.creatorJakob; Espinoza
dc.creatorDiego A.; Sellers
dc.creatorStephanie E.; Townsley
dc.creatorDanielle M.; Young
dc.creatorNeal S.; Bryceson
dc.creatorYenan T.; Dunbar
dc.creatorCynthia E.; Winkler
dc.creatorThomas
dc.date2017
dc.dateabr
dc.date2017-11-13T11:30:55Z
dc.date2017-11-13T11:30:55Z
dc.date.accessioned2018-03-29T05:45:58Z
dc.date.available2018-03-29T05:45:58Z
dc.identifierBlood. Amer Soc Hematology, v. 129, p. 1940 - 1946, 2017.
dc.identifier0006-4971
dc.identifier1528-0020
dc.identifierWOS:000398774000014
dc.identifier10.1182/blood-2016-08-734285
dc.identifierhttp://www.bloodjournal.org/content/129/14/1940?sso-checked=true
dc.identifierhttp://repositorio.unicamp.br/jspui/handle/REPOSIP/325824
dc.identifier.urihttp://repositorioslatinoamericanos.uchile.cl/handle/2250/1362830
dc.descriptionNatural killer (NK) cells have long been considered short-lived effectors of innate immunity. However, recent animal models and human studies suggest that subsets of NK cells have adaptive features. We investigate clonal relationships of various NK-cell subsets, including the adaptive population, by taking advantage of naturally occurring X-linked somatic PIGA mutations in hematopoietic stem and progenitor cells (HSPCs) from patients with paroxysmal nocturnal hemoglobinuria (PNH). The affected HSPCs and their progeny lack expression of glycosylphosphatidylinositol (GPI) anchors on their cell surface, allowing quantification of PIGA-mutant (GPI-negative) HSPC-derived peripheral blood cell populations. The fraction of GPI-negative cells within the CD56(dim) NK cells was markedly lower than that of neutrophils and the CD56(bright) NK-cell compartments. This discrepancy was most prominent within the adaptive CD56(dim) NK-cell population lacking PLZF expression. The functional properties of these adaptive NK cells were similar in PNH patients and healthy individuals. Our findings support the existence of a long-lived, adaptive NK-cell population maintained independently from GPI(pos)CD56(dim).
dc.description129
dc.description14
dc.description1940
dc.description1946
dc.descriptionNational Institutes of Health National Heart, Lung, and Blood Institute
dc.descriptionEuropean Research Council under the European Union [311335]
dc.descriptionSwedish Research Council
dc.descriptionNorwegian Research Council
dc.descriptionSwedish Foundation for Strategic Research
dc.descriptionWallenberg Foundation
dc.descriptionSwedish Cancer Foundation
dc.descriptionSwedish Childhood Cancer Foundation
dc.descriptionStockholm County Council
dc.descriptionKarolinska Institutet Center for Innovative Medicine
dc.languageEnglish
dc.publisherAmer Soc Hematology
dc.publisherWashington
dc.relationBlood
dc.rightsfechado
dc.sourceWOS
dc.titleAcquired Somatic Mutations In Pnh Reveal Long-term Maintenance Of Adaptive Nk Cells Independent Of Hspcs
dc.typeArtículos de revistas


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