1 Ocak 2023 Dergi makalesi Açık Erişim

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

Citation Style Language JSON

{
  "DOI": "10.1016/j.clim.2023.109691", 
  "abstract": "<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>", 
  "author": [
    {
      "family": "Erdem", 
      "given": " Serife"
    }, 
    {
      "family": "Haskologlu", 
      "given": " Sule"
    }, 
    {
      "family": "Haliloglu", 
      "given": " Yesim"
    }, 
    {
      "family": "Celikzencir", 
      "given": " Huriye"
    }, 
    {
      "family": "Arik", 
      "given": " Elif"
    }, 
    {
      "family": "Keskin", 
      "given": " Ozlem"
    }, 
    {
      "family": "Eltan", 
      "given": " Sevgi Bilgic"
    }, 
    {
      "family": "Yucel", 
      "given": " Esra"
    }, 
    {
      "family": "Canatan", 
      "given": " Halit"
    }, 
    {
      "family": "Avcilar", 
      "given": " Huseyin"
    }, 
    {
      "family": "Yilmaz", 
      "given": " Ebru"
    }, 
    {
      "family": "Ozcan", 
      "given": " Alper"
    }, 
    {
      "family": "Unal", 
      "given": " Ekrem"
    }, 
    {
      "family": "Karakukcu", 
      "given": " Musa"
    }, 
    {
      "family": "Celiksoy", 
      "given": " Mehmet Halil"
    }, 
    {
      "family": "Kilic", 
      "given": " Sara Sebnem"
    }, 
    {
      "family": "Demir", 
      "given": " Ayca"
    }, 
    {
      "family": "Genel", 
      "given": " Ferah"
    }, 
    {
      "family": "Gulez", 
      "given": " Nesrin"
    }, 
    {
      "family": "Koker", 
      "given": " Mustafa Yavuz"
    }
  ], 
  "container_title": "CLINICAL IMMUNOLOGY", 
  "id": "268622", 
  "issued": {
    "date-parts": [
      [
        2023, 
        1, 
        1
      ]
    ]
  }, 
  "page": "11", 
  "title": "Defective Treg generation and increased type 3 immune response in leukocyte adhesion deficiency 1", 
  "type": "article-journal", 
  "volume": "253"
}