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|a 10.1016/j.clim.2023.109691
|2 doi
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|a pubmed24n1197.xml
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|a (DE-627)NLM359343929
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|a (NLM)37433423
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|a (PII)S1521-6616(23)00454-0
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|a DE-627
|b ger
|c DE-627
|e rakwb
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|a eng
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|a Erdem, Serife
|e verfasserin
|4 aut
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|a Defective Treg generation and increased type 3 immune response in leukocyte adhesion deficiency 1
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|c 2023
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|a Text
|b txt
|2 rdacontent
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|a ƒaComputermedien
|b c
|2 rdamedia
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|a ƒa Online-Ressource
|b cr
|2 rdacarrier
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|a Date Completed 26.07.2023
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|a Date Revised 26.07.2023
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|a published: Print-Electronic
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|a Citation Status MEDLINE
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|a Copyright © 2023 Elsevier Inc. All rights reserved.
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|a 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 naïve 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 naïve T cells and elevated Th17s, and ILC3s in LAD-1 patients' peripheral blood suggest a type 3-skewed immunity and may contribute to LAD-1-associated autoimmune symptoms
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|a Journal Article
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|a Research Support, Non-U.S. Gov't
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|a ILC
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|a LAD-1
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|a Leukocyte adhesion deficiency
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|a Th17
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|a Treg
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|a Haskologlu, Sule
|e verfasserin
|4 aut
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|a Haliloglu, Yesim
|e verfasserin
|4 aut
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|a Celikzencir, Huriye
|e verfasserin
|4 aut
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|a Arik, Elif
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|4 aut
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|a Keskin, Ozlem
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|4 aut
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|a Eltan, Sevgi Bilgic
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|4 aut
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|a Yucel, Esra
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|4 aut
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|a Canatan, Halit
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|4 aut
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|a Avcilar, Huseyin
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|a Yilmaz, Ebru
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|a Ozcan, Alper
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|a Unal, Ekrem
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|a Karakukcu, Musa
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|a Celiksoy, Mehmet Halil
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|a Kilic, Sara Sebnem
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|a Demir, Ayca
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|a Genel, Ferah
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|a Gulez, Nesrin
|e verfasserin
|4 aut
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|a Koker, Mustafa Yavuz
|e verfasserin
|4 aut
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|a Ozen, Ahmet Oguzhan
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|4 aut
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|a Baris, Safa
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|a Metin, Ayse
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|a Guner, Sukru Nail
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|a Reisli, Ismail
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|a Keles, Sevgi
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|4 aut
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|a Dogu, Esin Figen
|e verfasserin
|4 aut
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|a Ikinciogullari, Kamile Aydan
|e verfasserin
|4 aut
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|a Eken, Ahmet
|e verfasserin
|4 aut
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|i Enthalten in
|t Clinical immunology (Orlando, Fla.)
|d 1999
|g 253(2023) vom: 30. Aug., Seite 109691
|w (DE-627)NLM098196855
|x 1521-7035
|7 nnns
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|g volume:253
|g year:2023
|g day:30
|g month:08
|g pages:109691
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|u http://dx.doi.org/10.1016/j.clim.2023.109691
|3 Volltext
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|a GBV_USEFLAG_A
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|a GBV_ILN_11
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|a GBV_ILN_350
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|a AR
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|d 253
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|h 109691
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