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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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<oai_dc:dc xmlns:dc="http://purl.org/dc/elements/1.1/" xmlns:oai_dc="http://www.openarchives.org/OAI/2.0/oai_dc/" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" xsi:schemaLocation="http://www.openarchives.org/OAI/2.0/oai_dc/ http://www.openarchives.org/OAI/2.0/oai_dc.xsd">
  <dc:creator>Erdem, Serife</dc:creator>
  <dc:creator>Haskologlu, Sule</dc:creator>
  <dc:creator>Haliloglu, Yesim</dc:creator>
  <dc:creator>Celikzencir, Huriye</dc:creator>
  <dc:creator>Arik, Elif</dc:creator>
  <dc:creator>Keskin, Ozlem</dc:creator>
  <dc:creator>Eltan, Sevgi Bilgic</dc:creator>
  <dc:creator>Yucel, Esra</dc:creator>
  <dc:creator>Canatan, Halit</dc:creator>
  <dc:creator>Avcilar, Huseyin</dc:creator>
  <dc:creator>Yilmaz, Ebru</dc:creator>
  <dc:creator>Ozcan, Alper</dc:creator>
  <dc:creator>Unal, Ekrem</dc:creator>
  <dc:creator>Karakukcu, Musa</dc:creator>
  <dc:creator>Celiksoy, Mehmet Halil</dc:creator>
  <dc:creator>Kilic, Sara Sebnem</dc:creator>
  <dc:creator>Demir, Ayca</dc:creator>
  <dc:creator>Genel, Ferah</dc:creator>
  <dc:creator>Gulez, Nesrin</dc:creator>
  <dc:creator>Koker, Mustafa Yavuz</dc:creator>
  <dc:date>2023-01-01</dc:date>
  <dc:description>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.</dc:description>
  <dc:identifier>https://aperta.ulakbim.gov.trrecord/268622</dc:identifier>
  <dc:identifier>oai:aperta.ulakbim.gov.tr:268622</dc:identifier>
  <dc:rights>info:eu-repo/semantics/openAccess</dc:rights>
  <dc:rights>http://www.opendefinition.org/licenses/cc-by</dc:rights>
  <dc:source>CLINICAL IMMUNOLOGY 253 11</dc:source>
  <dc:title>Defective Treg generation and increased type 3 immune response in leukocyte adhesion deficiency 1</dc:title>
  <dc:type>info:eu-repo/semantics/article</dc:type>
  <dc:type>publication-article</dc:type>
</oai_dc:dc>
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