Defective Treg generation and increased type 3 immune response in leukocyte adhesion deficiency 1
 
Yazarlar (25)
Yeşim Haliloglu
Huriye Çelikzencir
Elif Arık
Türkiye
Sevgi Bilgiç Ertan
Musa Karakükcü Erciyes Üniversitesi, Türkiye
Mehmet Halil Çeliksoy
Türkiye
Şebnem Sara Kılıç
Ferah Genel
Türkiye
Nesrin Gülez
Türkiye
Makale Türü Özgün Makale (SSCI, AHCI, SCI, SCI-Exp dergilerinde yayınlanan tam makale)
Dergi Adı Elsevier BV (Q1)
Dergi ISSN 1521-6616 Wos Dergi Scopus Dergi
Dergi Tarandığı Indeksler SCI-Expanded
Makale Dili Türkçe Basım Tarihi 08-2023
Cilt / Sayı / Sayfa 253 / 0 / – DOI 10.1016/j.clim.2023.109691
Makale Linki http://dx.doi.org/10.1016/j.clim.2023.109691
Özet
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.
Anahtar Kelimeler
Leukocyte adhesion deficiency | LAD-1 | Th17 | ILC | Treg
BM Sürdürülebilir Kalkınma Amaçları
Atıf Sayıları
Web of Science 5
Defective Treg generation and increased type 3 immune response in leukocyte adhesion deficiency 1

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