ILC3 deficiency and generalized ILC abnormalities in DOCK8-deficient patients

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dc.authorid0000-0002-2691-4826
dc.authorid0000-0001-7439-2762
dc.contributor.authorEken, Ahmet
dc.contributor.authorCansever, Murat
dc.contributor.authorOkuş, Fatma Zehra
dc.contributor.authorErdem, Şerife
dc.contributor.authorNain, Ercan
dc.contributor.authorAzizoğlu, Zehra Betül
dc.contributor.authorHaliloğlu, Yeşim
dc.contributor.authorKarakukçu, Musa
dc.contributor.authorÖzcan, Alper
dc.contributor.authorDevecioğlu, Ömer
dc.contributor.authorAksu, Güzide
dc.contributor.authorAyyıldız, Zeynep Arıkan
dc.contributor.authorTopal, Erdem
dc.contributor.authorAydıner, Elif Karakoç
dc.contributor.authorKıykım, Ayça
dc.contributor.authorMetin, Ayşe
dc.contributor.authorÇipe, Funda
dc.contributor.authorKaya, Ayşenur
dc.contributor.authorArtaç, Hasibe
dc.contributor.authorReisli, İsmail
dc.contributor.authorGüner, Şükrü
dc.contributor.authorUygun, Vedat
dc.contributor.authorKarasu, Gülsün Tezcan
dc.contributor.authorAltuntaş, Hamiyet Dönmez
dc.contributor.authorCanatan, Halit
dc.contributor.authorOukka, Mohamed
dc.contributor.authorÖzen, Ahmet
dc.contributor.authorChatila, Talal A.
dc.contributor.authorKeleş, Sevgi
dc.contributor.authorBarış, Sefa
dc.contributor.authorÜnal, Ekrem
dc.contributor.authorPatıroğlu, Türkan
dc.date.accessioned2020-03-26T20:19:55Z
dc.date.available2020-03-26T20:19:55Z
dc.date.issued2019
dc.departmentSelçuk Üniversitesi, Tıp Fakültesi, Dahili Tıp Bilimleri Bölümüen_US
dc.description.abstractBackground: Dedicator of cytokinesis 8 (DOCK8) deficiency is the main cause of the autosomal recessive hyper-IgE syndrome (HIES). We previously reported the selective loss of group 3 innate lymphoid cell (ILC) number and function in a Dock8-deficient mouse model. In this study, we sought to test whether DOCK8 is required for the function and maintenance of ILC subsets in humans. Methods: Peripheral blood ILC1-3 subsets of 16 DOCK8-deficient patients recruited at the pretransplant stage, and seven patients with autosomal dominant (AD) HIES due to STAT3 mutations, were compared with those of healthy controls or post-transplant DOCK8-deficient patients (n = 12) by flow cytometry and real-time qPCR. Sorted total ILCs from DOCK8- or STAT3-mutant patients and healthy controls were assayed for survival, apoptosis, proliferation, and activation by IL-7, IL-23, and IL-12 by cell culture, flow cytometry, and phospho-flow assays. Results: DOCK8-deficient but not STAT3-mutant patients exhibited a profound depletion of ILC3s, and to a lesser extent ILC2s, in their peripheral blood. DOCK8-deficient ILC1-3 subsets had defective proliferation, expressed lower levels of IL-7R, responded less to IL-7, IL-12, or IL-23 cytokines, and were more prone to apoptosis compared with those of healthy controls. Conclusion: DOCK8 regulates human ILC3 expansion and survival, and more globally ILC cytokine signaling and proliferation. DOCK8 deficiency leads to loss of ILC3 from peripheral blood. ILC3 deficiency may contribute to the susceptibility of DOCK8-deficient patients to infections. © 2019 EAACI and John Wiley and Sons A/S. Published by John Wiley and Sons Ltd.en_US
dc.description.sponsorshipTOA?2016?6130 National Institutes of Health: R01AI128976, 5R01AI085090 315S315, 215S725en_US
dc.description.sponsorshipThis work was supported partly by the Erciyes University BAP grant, TOA?2016?6130; TUBITAK grants, 215S725 and 315S315 to Ahmet Eken; and National Institutes of Health grant 5R01AI085090 and R01AI128976 to Talal A. Chatila.en_US
dc.identifier.citationEken, A., Cansever, M., Okus, F. Z., Erdem, S., Nain, E., Azizoglu, Z. B., Haliloglu, Y., Karakukcu, M., Ozcan, A., Devecioglu, O., Aksu, G., Ayyildiz, Z. A., Topal, E., Aydiner, E. K., Kiykim, A., Metin, A., Cipe, F., Kaya, A., Artac, H., Reisli, I., Guner, S. N., Uygun, V., Karasu, G., Altuntas, H. D., Canatan, H., Oukka, M., Ozen, A., Chatila, T. A., Keles, S., Baris, S., Unal, E., Patiroglu, T. (2019). ILC3 Deficiency and Generalized ILC Abnormalities in DOCK8-Deficient Patients. Allergy: European Journal of Allergy and Clinical Immunology, -(-), 921-932.
dc.identifier.doi10.1111/all.14081en_US
dc.identifier.issn0105-4538en_US
dc.identifier.pmid31596517en_US
dc.identifier.scopusqualityQ1en_US
dc.identifier.urihttps://dx.doi.org/10.1111/all.14081
dc.identifier.urihttps://hdl.handle.net/20.500.12395/38448
dc.identifier.wosWOS:000529134100017en_US
dc.identifier.wosqualityQ1en_US
dc.indekslendigikaynakWeb of Scienceen_US
dc.indekslendigikaynakScopusen_US
dc.indekslendigikaynakPubMeden_US
dc.institutionauthorArtac H.
dc.language.isoenen_US
dc.publisherBlackwell Publishing Ltden_US
dc.relation.ispartofAllergy: European Journal of Allergy and Clinical Immunologyen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/closedAccessen_US
dc.selcuk20240510_oaigen_US
dc.subjectDOCK8en_US
dc.subjectHyper-IgE syndrome (HIES)en_US
dc.subjectILCen_US
dc.subjectILC3en_US
dc.subjectSTAT3en_US
dc.titleILC3 deficiency and generalized ILC abnormalities in DOCK8-deficient patientsen_US
dc.typeArticleen_US

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