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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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  <identifier identifierType="URL">https://aperta.ulakbim.gov.tr/record/268622</identifier>
  <creators>
    <creator>
      <creatorName>Erdem, Serife</creatorName>
      <givenName>Serife</givenName>
      <familyName>Erdem</familyName>
    </creator>
    <creator>
      <creatorName>Haskologlu, Sule</creatorName>
      <givenName>Sule</givenName>
      <familyName>Haskologlu</familyName>
      <affiliation>Ankara Univ, Dept Pediat Allergy &amp; Immunol, Sch Med, Ankara, Turkiye</affiliation>
    </creator>
    <creator>
      <creatorName>Haliloglu, Yesim</creatorName>
      <givenName>Yesim</givenName>
      <familyName>Haliloglu</familyName>
    </creator>
    <creator>
      <creatorName>Celikzencir, Huriye</creatorName>
      <givenName>Huriye</givenName>
      <familyName>Celikzencir</familyName>
      <affiliation>Erciyes Univ, Dept Immunol, Sch Med, Kayseri, Turkiye</affiliation>
    </creator>
    <creator>
      <creatorName>Arik, Elif</creatorName>
      <givenName>Elif</givenName>
      <familyName>Arik</familyName>
      <affiliation>Gaziantep Univ, Dept Pediat Allergy &amp; Immunol, Sch Med, Gaziantep, Turkiye</affiliation>
    </creator>
    <creator>
      <creatorName>Keskin, Ozlem</creatorName>
      <givenName>Ozlem</givenName>
      <familyName>Keskin</familyName>
      <affiliation>Gaziantep Univ, Dept Pediat Allergy &amp; Immunol, Sch Med, Gaziantep, Turkiye</affiliation>
    </creator>
    <creator>
      <creatorName>Eltan, Sevgi Bilgic</creatorName>
      <givenName>Sevgi Bilgic</givenName>
      <familyName>Eltan</familyName>
      <affiliation>Marmara Univ, Dept Pediat Allergy &amp; Immunol, Sch Med, Istanbul, Turkiye</affiliation>
    </creator>
    <creator>
      <creatorName>Yucel, Esra</creatorName>
      <givenName>Esra</givenName>
      <familyName>Yucel</familyName>
      <affiliation>Istanbul Univ, Dept Pediat Allergy &amp; Immunol, Sch Med, Istanbul, Turkiye</affiliation>
    </creator>
    <creator>
      <creatorName>Canatan, Halit</creatorName>
      <givenName>Halit</givenName>
      <familyName>Canatan</familyName>
    </creator>
    <creator>
      <creatorName>Avcilar, Huseyin</creatorName>
      <givenName>Huseyin</givenName>
      <familyName>Avcilar</familyName>
      <affiliation>Erciyes Univ, Betul Ziya Eren Genome &amp; Stem Cell Ctr, Kayseri, Turkiye</affiliation>
    </creator>
    <creator>
      <creatorName>Yilmaz, Ebru</creatorName>
      <givenName>Ebru</givenName>
      <familyName>Yilmaz</familyName>
      <affiliation>Erciyes Univ, Dept Pediat Hematol &amp; Oncol, Sch Med, Kayseri, Turkiye</affiliation>
    </creator>
    <creator>
      <creatorName>Ozcan, Alper</creatorName>
      <givenName>Alper</givenName>
      <familyName>Ozcan</familyName>
      <affiliation>Erciyes Univ, Dept Pediat Hematol &amp; Oncol, Sch Med, Kayseri, Turkiye</affiliation>
    </creator>
    <creator>
      <creatorName>Unal, Ekrem</creatorName>
      <givenName>Ekrem</givenName>
      <familyName>Unal</familyName>
    </creator>
    <creator>
      <creatorName>Karakukcu, Musa</creatorName>
      <givenName>Musa</givenName>
      <familyName>Karakukcu</familyName>
      <affiliation>Erciyes Univ, Dept Pediat Hematol &amp; Oncol, Sch Med, Kayseri, Turkiye</affiliation>
    </creator>
    <creator>
      <creatorName>Celiksoy, Mehmet Halil</creatorName>
      <givenName>Mehmet Halil</givenName>
      <familyName>Celiksoy</familyName>
      <affiliation>Univ Hlth Sci, Istanbul Basaksehir Cam &amp; Sakura City Hosp, Pediat Allergy &amp; Immunol Clin, Istanbul, Turkiye</affiliation>
    </creator>
    <creator>
      <creatorName>Kilic, Sara Sebnem</creatorName>
      <givenName>Sara Sebnem</givenName>
      <familyName>Kilic</familyName>
      <affiliation>Bursa Uludag Univ, Sch Med, Dept Pediat Immunol &amp; Rheumatol, Bursa, Turkiye</affiliation>
    </creator>
    <creator>
      <creatorName>Demir, Ayca</creatorName>
      <givenName>Ayca</givenName>
      <familyName>Demir</familyName>
    </creator>
    <creator>
      <creatorName>Genel, Ferah</creatorName>
      <givenName>Ferah</givenName>
      <familyName>Genel</familyName>
    </creator>
    <creator>
      <creatorName>Gulez, Nesrin</creatorName>
      <givenName>Nesrin</givenName>
      <familyName>Gulez</familyName>
      <affiliation>Univ Hlth Sci, Dr Behcet Uz Childrens Educ &amp; Res Hosp, Dept Pediat Allergy &amp; Immunol, Izmir, Turkiye</affiliation>
    </creator>
    <creator>
      <creatorName>Koker, Mustafa Yavuz</creatorName>
      <givenName>Mustafa Yavuz</givenName>
      <familyName>Koker</familyName>
      <affiliation>Erciyes Univ, Dept Immunol, Sch Med, Kayseri, Turkiye</affiliation>
    </creator>
  </creators>
  <titles>
    <title>Defective Treg Generation And Increased Type 3 Immune Response In Leukocyte Adhesion Deficiency 1</title>
  </titles>
  <publisher>Aperta</publisher>
  <publicationYear>2023</publicationYear>
  <dates>
    <date dateType="Issued">2023-01-01</date>
  </dates>
  <resourceType resourceTypeGeneral="Text">Journal article</resourceType>
  <alternateIdentifiers>
    <alternateIdentifier alternateIdentifierType="url">https://aperta.ulakbim.gov.tr/record/268622</alternateIdentifier>
  </alternateIdentifiers>
  <relatedIdentifiers>
    <relatedIdentifier relatedIdentifierType="DOI" relationType="IsIdenticalTo">10.1016/j.clim.2023.109691</relatedIdentifier>
  </relatedIdentifiers>
  <rightsList>
    <rights rightsURI="http://www.opendefinition.org/licenses/cc-by">Creative Commons Attribution</rights>
    <rights rightsURI="info:eu-repo/semantics/openAccess">Open Access</rights>
  </rightsList>
  <descriptions>
    <description descriptionType="Abstract">&lt;p&gt;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.&lt;/p&gt;</description>
  </descriptions>
</resource>
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