دورية أكاديمية
Defective Treg generation and increased type 3 immune response in leukocyte adhesion deficiency 1.
| العنوان: | Defective Treg generation and increased type 3 immune response in leukocyte adhesion deficiency 1. |
|---|---|
| المؤلفون: | Erdem S; Erciyes University School of Medicine, Department of Medical Biology, Kayseri, Turkey; Erciyes University, Betul-Ziya Eren Genome and Stem Cell Center, Kayseri, Turkey., Haskologlu S; Ankara University School of Medicine, Department of Pediatric Allergy and Immunology, Ankara, Turkey., Haliloglu Y; Erciyes University School of Medicine, Department of Medical Biology, Kayseri, Turkey; Erciyes University, Betul-Ziya Eren Genome and Stem Cell Center, Kayseri, Turkey., Celikzencir H; Erciyes University School of Medicine, Department of Immunology, Kayseri, Turkey., Arik E; Gaziantep University School of Medicine, Department of Pediatric Allergy and Immunology, Gaziantep, Turkey., Keskin O; Gaziantep University School of Medicine, Department of Pediatric Allergy and Immunology, Gaziantep, Turkey., Eltan SB; Marmara University School of Medicine, Department of Pediatric Allergy and Immunology, Istanbul, Turkey., Yucel E; Istanbul University School of Medicine, Department of Pediatric Allergy and Immunology, Istanbul, Turkey., Canatan H; Erciyes University School of Medicine, Department of Medical Biology, Kayseri, Turkey; Erciyes University, Betul-Ziya Eren Genome and Stem Cell Center, Kayseri, Turkey., Avcilar H; Erciyes University, Betul-Ziya Eren Genome and Stem Cell Center, Kayseri, Turkey., Yilmaz E; Erciyes University School of Medicine, Department of Pediatric Hematology and Oncology, Kayseri, Turkey., Ozcan A; Erciyes University School of Medicine, Department of Pediatric Hematology and Oncology, Kayseri, Turkey., Unal E; Erciyes University, Betul-Ziya Eren Genome and Stem Cell Center, Kayseri, Turkey; Erciyes University School of Medicine, Department of Pediatric Hematology and Oncology, Kayseri, Turkey., Karakukcu M; Erciyes University School of Medicine, Department of Pediatric Hematology and Oncology, Kayseri, Turkey., Celiksoy MH; İstanbul Başakşehir Çam ve Sakura City Hospital, University of Health Sciences, Pediatric Allergy and Immunology Clinic, Istanbul, Turkey., Kilic SS; Bursa Uludag University, School of Medicine, Department of Pediatric Immunology and Rheumatology, Bursa, Turkey., Demir A; Dr Behcet Uz Children's Education and Research Hospital, University of Health Sciences, Department of Pediatric Allergy and Immunology, Izmir, Turkey., Genel F; Dr Behcet Uz Children's Education and Research Hospital, University of Health Sciences, Department of Pediatric Allergy and Immunology, Izmir, Turkey., Gulez N; Dr Behcet Uz Children's Education and Research Hospital, University of Health Sciences, Department of Pediatric Allergy and Immunology, Izmir, Turkey., Koker MY; Erciyes University School of Medicine, Department of Immunology, Kayseri, Turkey., Ozen AO; Marmara University School of Medicine, Department of Pediatric Allergy and Immunology, Istanbul, Turkey., Baris S; Marmara University School of Medicine, Department of Pediatric Allergy and Immunology, Istanbul, Turkey., Metin A; Ankara City Hospital, University of Health Sciences, Department of Pediatric Allergy and Immunology, Ankara, Turkey., Guner SN; Necmettin Erbakan University School of Medicine, Department of Pediatric Allergy and Immunology, Konya, Turkey., Reisli I; Necmettin Erbakan University School of Medicine, Department of Pediatric Allergy and Immunology, Konya, Turkey., Keles S; Necmettin Erbakan University School of Medicine, Department of Pediatric Allergy and Immunology, Konya, Turkey., Dogu EF; Ankara University School of Medicine, Department of Pediatric Allergy and Immunology, Ankara, Turkey., Ikinciogullari KA; Ankara University School of Medicine, Department of Pediatric Allergy and Immunology, Ankara, Turkey., Eken A; Erciyes University School of Medicine, Department of Medical Biology, Kayseri, Turkey; Erciyes University, Betul-Ziya Eren Genome and Stem Cell Center, Kayseri, Turkey. Electronic address: ahmet.eken@gmail.com. |
| المصدر: | Clinical immunology (Orlando, Fla.) [Clin Immunol] 2023 Aug; Vol. 253, pp. 109691. Date of Electronic Publication: 2023 Jul 09. |
| نوع المنشور: | Journal Article; Research Support, Non-U.S. Gov't |
| اللغة: | English |
| بيانات الدورية: | Publisher: Academic Press Country of Publication: United States NLM ID: 100883537 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1521-7035 (Electronic) Linking ISSN: 15216616 NLM ISO Abbreviation: Clin Immunol Subsets: MEDLINE |
| أسماء مطبوعة: | Original Publication: Orlando, FL : Academic Press, c1999- |
| مواضيع طبية MeSH: | T-Lymphocytes, Regulatory* , Leukocyte-Adhesion Deficiency Syndrome*, Humans ; Immunity, Innate ; CD4-Positive T-Lymphocytes ; Th17 Cells |
| مستخلص: | 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. Competing Interests: Declaration of Competing Interest The authors declare no competing interest. (Copyright © 2023 Elsevier Inc. All rights reserved.) |
| فهرسة مساهمة: | Keywords: ILC; LAD-1; Leukocyte adhesion deficiency; Th17; Treg |
| SCR Disease Name: | Leukocyte adhesion deficiency type 1 |
| تواريخ الأحداث: | Date Created: 20230711 Date Completed: 20230726 Latest Revision: 20230726 |
| رمز التحديث: | 20231215 |
| DOI: | 10.1016/j.clim.2023.109691 |
| PMID: | 37433423 |
| قاعدة البيانات: | MEDLINE |
Full Text via ClinicalKey 
Full Text Finder | تدمد: | 1521-7035 |
|---|---|
| DOI: | 10.1016/j.clim.2023.109691 |