Defective Treg generation and increased type 3 immune response in leukocyte adhesion deficiency 1

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dc.contributor.authorErdem, Serife
dc.contributor.authorHaskologlu, Sule
dc.contributor.authorHaliloglu, Yesim
dc.contributor.authorCelikzencir, Huriye
dc.contributor.authorArik, Elif
dc.contributor.authorKeskin, Ozlem
dc.contributor.authorEltan, Sevgi Bilgic
dc.date.accessioned2024-02-23T14:02:28Z
dc.date.available2024-02-23T14:02:28Z
dc.date.issued2023
dc.departmentNEÜen_US
dc.description.abstractIn 15 Turkish LAD-1 patients and controls, we assessed the impact of pathogenic ITGB2 mutations on Th17/Treg differentiation and functions, and innate lymphoid cell (ILC) subsets. The percentage of peripheral blood Treg cells, in vitro-generated induced Tregs differentiated from naive CD4+ T cells were decreased despite the elevated absolute counts of CD4+ cells in LAD-1 patients. Serum IL-23 levels were elevated in LAD-1 patients. Post-curdlan stimulation, LAD-1 patient-derived PBMCs produced more IL-17A. Additionally, the percentages of CD18-deficient Th17 cells expanded from total or naive CD4+ T cells were higher. The blood ILC3 subset was significantly elevated in LAD-1. Finally, LAD-1 PBMCs showed defects in trans-well migration and proliferation and were more resistant to apoptosis. Defects in de novo generation of Tregs from CD18-deficient naive T cells and elevated Th17s, and ILC3s in LAD1 patients' peripheral blood suggest a type 3-skewed immunity and may contribute to LAD-1-associated autoimmune symptoms.en_US
dc.description.sponsorshipErciyes University BAP grant [TSG-2023-10935]; TUBITAK grant [221S883]; BAGEP 2022 Grant by Science Academy; Turkish Academy of Sciencesen_US
dc.description.sponsorshipThis work was supported partly by the Erciyes University BAP grant (TSG-2023-10935) ; and by the TUBITAK grant (#221S883) , BAGEP 2022 Grant by Science Academy, and TUBA GEBIP 2021 grant by Turkish Academy of Sciences to AE.en_US
dc.identifier.doi10.1016/j.clim.2023.109691
dc.identifier.issn1521-6616
dc.identifier.issn1521-7035
dc.identifier.pmid37433423en_US
dc.identifier.scopus2-s2.0-85165054835en_US
dc.identifier.scopusqualityQ1en_US
dc.identifier.urihttps://doi.org/10.1016/j.clim.2023.109691
dc.identifier.urihttps://hdl.handle.net/20.500.12452/11726
dc.identifier.volume253en_US
dc.identifier.wosWOS:001044455300001en_US
dc.indekslendigikaynakWeb of Scienceen_US
dc.indekslendigikaynakScopusen_US
dc.indekslendigikaynakPubMeden_US
dc.language.isoenen_US
dc.publisherAcademic Press Inc Elsevier Scienceen_US
dc.relation.ispartofClinical Immunologyen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/closedAccessen_US
dc.subjectLeukocyte Adhesion Deficiencyen_US
dc.subjectLad-1en_US
dc.subjectTh17en_US
dc.subjectIlcen_US
dc.subjectTregen_US
dc.titleDefective Treg generation and increased type 3 immune response in leukocyte adhesion deficiency 1en_US
dc.typeArticleen_US

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