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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
<?xml version='1.0' encoding='utf-8'?> <resource xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" xmlns="http://datacite.org/schema/kernel-4" xsi:schemaLocation="http://datacite.org/schema/kernel-4 http://schema.datacite.org/meta/kernel-4.1/metadata.xsd"> <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 & 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 & Immunol, Sch Med, Gaziantep, Turkiye</affiliation> </creator> <creator> <creatorName>Keskin, Ozlem</creatorName> <givenName>Ozlem</givenName> <familyName>Keskin</familyName> <affiliation>Gaziantep Univ, Dept Pediat Allergy & 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 & Immunol, Sch Med, Istanbul, Turkiye</affiliation> </creator> <creator> <creatorName>Yucel, Esra</creatorName> <givenName>Esra</givenName> <familyName>Yucel</familyName> <affiliation>Istanbul Univ, Dept Pediat Allergy & 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 & Stem Cell Ctr, Kayseri, Turkiye</affiliation> </creator> <creator> <creatorName>Yilmaz, Ebru</creatorName> <givenName>Ebru</givenName> <familyName>Yilmaz</familyName> <affiliation>Erciyes Univ, Dept Pediat Hematol & Oncol, Sch Med, Kayseri, Turkiye</affiliation> </creator> <creator> <creatorName>Ozcan, Alper</creatorName> <givenName>Alper</givenName> <familyName>Ozcan</familyName> <affiliation>Erciyes Univ, Dept Pediat Hematol & 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 & 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 & Sakura City Hosp, Pediat Allergy & 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 & 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 & Res Hosp, Dept Pediat Allergy & 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"><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></description> </descriptions> </resource>
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