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

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

Copyright © 2023 Elsevier Inc. All rights reserved.

Bibliographische Detailangaben
Veröffentlicht in:Clinical immunology (Orlando, Fla.). - 1999. - 253(2023) vom: 30. Aug., Seite 109691
1. Verfasser: Erdem, Serife (VerfasserIn)
Weitere Verfasser: Haskologlu, Sule, Haliloglu, Yesim, Celikzencir, Huriye, Arik, Elif, Keskin, Ozlem, Eltan, Sevgi Bilgic, Yucel, Esra, Canatan, Halit, Avcilar, Huseyin, Yilmaz, Ebru, Ozcan, Alper, Unal, Ekrem, Karakukcu, Musa, Celiksoy, Mehmet Halil, Kilic, Sara Sebnem, Demir, Ayca, Genel, Ferah, Gulez, Nesrin, Koker, Mustafa Yavuz, Ozen, Ahmet Oguzhan, Baris, Safa, Metin, Ayse, Guner, Sukru Nail, Reisli, Ismail, Keles, Sevgi, Dogu, Esin Figen, Ikinciogullari, Kamile Aydan, Eken, Ahmet
Format: Online-Aufsatz
Sprache:English
Veröffentlicht: 2023
Zugriff auf das übergeordnete Werk:Clinical immunology (Orlando, Fla.)
Schlagworte:Journal Article Research Support, Non-U.S. Gov't ILC LAD-1 Leukocyte adhesion deficiency Th17 Treg
Beschreibung
Zusammenfassung:Copyright © 2023 Elsevier Inc. All rights reserved.
In 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 naïve 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 naïve T cells and elevated Th17s, and ILC3s in LAD-1 patients' peripheral blood suggest a type 3-skewed immunity and may contribute to LAD-1-associated autoimmune symptoms
Beschreibung:Date Completed 26.07.2023
Date Revised 26.07.2023
published: Print-Electronic
Citation Status MEDLINE
ISSN:1521-7035
DOI:10.1016/j.clim.2023.109691