| 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 |
| Atıf Sayıları | |
| Web of Science | 5 |
| Dergi Adı | CLINICAL IMMUNOLOGY |
| Yayıncı | Academic Press Inc. |
| Açık Erişim | Hayır |
| ISSN | 1521-6616 |
| E-ISSN | 1521-7035 |
| CiteScore | 7,1 |
| SJR | 1,279 |
| SNIP | 0,850 |