Dergi makalesi Açık Erişim

Defective Treg generation and increased type 3 immune response in leukocyte adhesion deficiency 1

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


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    "creators": [
      {
        "name": "Erdem, Serife"
      }, 
      {
        "affiliation": "Ankara Univ, Dept Pediat Allergy & Immunol, Sch Med, Ankara, Turkiye", 
        "name": "Haskologlu, Sule"
      }, 
      {
        "name": "Haliloglu, Yesim"
      }, 
      {
        "affiliation": "Erciyes Univ, Dept Immunol, Sch Med, Kayseri, Turkiye", 
        "name": "Celikzencir, Huriye"
      }, 
      {
        "affiliation": "Gaziantep Univ, Dept Pediat Allergy & Immunol, Sch Med, Gaziantep, Turkiye", 
        "name": "Arik, Elif"
      }, 
      {
        "affiliation": "Gaziantep Univ, Dept Pediat Allergy & Immunol, Sch Med, Gaziantep, Turkiye", 
        "name": "Keskin, Ozlem"
      }, 
      {
        "affiliation": "Marmara Univ, Dept Pediat Allergy & Immunol, Sch Med, Istanbul, Turkiye", 
        "name": "Eltan, Sevgi Bilgic"
      }, 
      {
        "affiliation": "Istanbul Univ, Dept Pediat Allergy & Immunol, Sch Med, Istanbul, Turkiye", 
        "name": "Yucel, Esra"
      }, 
      {
        "name": "Canatan, Halit"
      }, 
      {
        "affiliation": "Erciyes Univ, Betul Ziya Eren Genome & Stem Cell Ctr, Kayseri, Turkiye", 
        "name": "Avcilar, Huseyin"
      }, 
      {
        "affiliation": "Erciyes Univ, Dept Pediat Hematol & Oncol, Sch Med, Kayseri, Turkiye", 
        "name": "Yilmaz, Ebru"
      }, 
      {
        "affiliation": "Erciyes Univ, Dept Pediat Hematol & Oncol, Sch Med, Kayseri, Turkiye", 
        "name": "Ozcan, Alper"
      }, 
      {
        "name": "Unal, Ekrem"
      }, 
      {
        "affiliation": "Erciyes Univ, Dept Pediat Hematol & Oncol, Sch Med, Kayseri, Turkiye", 
        "name": "Karakukcu, Musa"
      }, 
      {
        "affiliation": "Univ Hlth Sci, Istanbul Basaksehir Cam & Sakura City Hosp, Pediat Allergy & Immunol Clin, Istanbul, Turkiye", 
        "name": "Celiksoy, Mehmet Halil"
      }, 
      {
        "affiliation": "Bursa Uludag Univ, Sch Med, Dept Pediat Immunol & Rheumatol, Bursa, Turkiye", 
        "name": "Kilic, Sara Sebnem"
      }, 
      {
        "name": "Demir, Ayca"
      }, 
      {
        "name": "Genel, Ferah"
      }, 
      {
        "affiliation": "Univ Hlth Sci, Dr Behcet Uz Childrens Educ & Res Hosp, Dept Pediat Allergy & Immunol, Izmir, Turkiye", 
        "name": "Gulez, Nesrin"
      }, 
      {
        "affiliation": "Erciyes Univ, Dept Immunol, Sch Med, Kayseri, Turkiye", 
        "name": "Koker, Mustafa Yavuz"
      }
    ], 
    "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>", 
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      "pages": "11", 
      "title": "CLINICAL IMMUNOLOGY", 
      "volume": "253"
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    "title": "Defective Treg generation and increased type 3 immune response in leukocyte adhesion deficiency 1"
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