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Erdem, Serife; 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
{ "@context": "https://schema.org/", "@id": 268622, "@type": "ScholarlyArticle", "creator": [ { "@type": "Person", "name": "Erdem, Serife" }, { "@type": "Person", "affiliation": "Ankara Univ, Dept Pediat Allergy & Immunol, Sch Med, Ankara, Turkiye", "name": "Haskologlu, Sule" }, { "@type": "Person", "name": "Haliloglu, Yesim" }, { "@type": "Person", "affiliation": "Erciyes Univ, Dept Immunol, Sch Med, Kayseri, Turkiye", "name": "Celikzencir, Huriye" }, { "@type": "Person", "affiliation": "Gaziantep Univ, Dept Pediat Allergy & Immunol, Sch Med, Gaziantep, Turkiye", "name": "Arik, Elif" }, { "@type": "Person", "affiliation": "Gaziantep Univ, Dept Pediat Allergy & Immunol, Sch Med, Gaziantep, Turkiye", "name": "Keskin, Ozlem" }, { "@type": "Person", "affiliation": "Marmara Univ, Dept Pediat Allergy & Immunol, Sch Med, Istanbul, Turkiye", "name": "Eltan, Sevgi Bilgic" }, { "@type": "Person", "affiliation": "Istanbul Univ, Dept Pediat Allergy & Immunol, Sch Med, Istanbul, Turkiye", "name": "Yucel, Esra" }, { "@type": "Person", "name": "Canatan, Halit" }, { "@type": "Person", "affiliation": "Erciyes Univ, Betul Ziya Eren Genome & Stem Cell Ctr, Kayseri, Turkiye", "name": "Avcilar, Huseyin" }, { "@type": "Person", "affiliation": "Erciyes Univ, Dept Pediat Hematol & Oncol, Sch Med, Kayseri, Turkiye", "name": "Yilmaz, Ebru" }, { "@type": "Person", "affiliation": "Erciyes Univ, Dept Pediat Hematol & Oncol, Sch Med, Kayseri, Turkiye", "name": "Ozcan, Alper" }, { "@type": "Person", "name": "Unal, Ekrem" }, { "@type": "Person", "affiliation": "Erciyes Univ, Dept Pediat Hematol & Oncol, Sch Med, Kayseri, Turkiye", "name": "Karakukcu, Musa" }, { "@type": "Person", "affiliation": "Univ Hlth Sci, Istanbul Basaksehir Cam & Sakura City Hosp, Pediat Allergy & Immunol Clin, Istanbul, Turkiye", "name": "Celiksoy, Mehmet Halil" }, { "@type": "Person", "affiliation": "Bursa Uludag Univ, Sch Med, Dept Pediat Immunol & Rheumatol, Bursa, Turkiye", "name": "Kilic, Sara Sebnem" }, { "@type": "Person", "name": "Demir, Ayca" }, { "@type": "Person", "name": "Genel, Ferah" }, { "@type": "Person", "affiliation": "Univ Hlth Sci, Dr Behcet Uz Childrens Educ & Res Hosp, Dept Pediat Allergy & Immunol, Izmir, Turkiye", "name": "Gulez, Nesrin" }, { "@type": "Person", "affiliation": "Erciyes Univ, Dept Immunol, Sch Med, Kayseri, Turkiye", "name": "Koker, Mustafa Yavuz" } ], "datePublished": "2023-01-01", "description": "<p>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 naive 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 naive T cells and elevated Th17s, and ILC3s in LAD1 patients' peripheral blood suggest a type 3-skewed immunity and may contribute to LAD-1-associated autoimmune symptoms.</p>", "headline": "Defective Treg generation and increased type 3 immune response in leukocyte adhesion deficiency 1", "identifier": 268622, "image": "https://aperta.ulakbim.gov.tr/static/img/logo/aperta_logo_with_icon.svg", "license": "http://www.opendefinition.org/licenses/cc-by", "name": "Defective Treg generation and increased type 3 immune response in leukocyte adhesion deficiency 1", "url": "https://aperta.ulakbim.gov.tr/record/268622" }
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