Publication: Defective Treg generation and increased type 3 immune response in leukocyte adhesion deficiency 1
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Date
2023-08-01
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Abstract
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.
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Tıp, Yaşam Bilimleri, Sağlık Bilimleri, Temel Bilimler, Medicine, Life Sciences, Health Sciences, Natural Sciences, Klinik Tıp (MED), Yaşam Bilimleri (LIFE), Klinik Tıp, İmmünoloji, ALERJİ, Clinical Medicine (MED), Life Sciences (LIFE), CLINICAL MEDICINE, IMMUNOLOGY, ALLERGY, İmmünoloji ve Alerji, Immunology and Allergy, Immunology, ILC, LAD-1, Leukocyte adhesion deficiency, Th17, Treg
Citation
Erdem S., Haskologlu S., Haliloglu Y., Celikzencir H., Arik E., Keskin O., Eltan S. B., Yucel E., Canatan H., Avcilar H., et al., "Defective Treg generation and increased type 3 immune response in leukocyte adhesion deficiency 1", Clinical Immunology, cilt.253, 2023