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231226s2022 xx |||||o 00| ||eng c |
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|a 10.1016/j.clim.2022.109098
|2 doi
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|a pubmed25n1149.xml
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|a (NLM)35973636
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|a (PII)S1521-6616(22)00179-6
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|a DE-627
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|e rakwb
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|a eng
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| 100 |
1 |
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|a Sasahara, Yoji
|e verfasserin
|4 aut
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| 245 |
1 |
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|a Impairment of cytokine production following immunological synapse formation in patients with Wiskott-Aldrich syndrome and leukocyte adhesion deficiency type 1
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|c 2022
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|a Text
|b txt
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|a ƒaComputermedien
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|a ƒa Online-Ressource
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|a Date Completed 13.09.2022
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|a Date Revised 11.11.2022
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|a published: Print-Electronic
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|a Citation Status MEDLINE
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|a Copyright © 2022 The Authors. Published by Elsevier Inc. All rights reserved.
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|a T cells following immunological synapse (IS) formation with antigen-presenting cells produce multiple cytokines through T cell receptor, integrin, and costimulatory signaling. Here, we investigated the cytokine profiles following IS formation in response to staphylococcal superantigen exposure in three adolescent patients with classical Wiskott-Aldrich syndrome (WAS) and in one patient with leukocyte adhesion deficiency (LAD) type 1. All WAS patients showed lower Th1 and Th2-skewed cytokine production; similar results were observed in the flow cytometric analysis of IFNγ- and IL-4-producing T cells. The patient with LAD type 1 with somatic mosaicism in 2% of CD8+ T cells showed lower Th1 and Th2 cytokine production than healthy controls. The patients with WAS were susceptible to infections and atopic manifestations, and the patients with LAD type 1 showed cold abscess on their skin, our findings using patient samples provide clinical insights into the mechanisms underlying immunodeficiency related to the symptoms of each disease
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|a Journal Article
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|a Research Support, Non-U.S. Gov't
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|a Immunological synapse
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| 650 |
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|a Integrin
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|a Leukocyte adhesion deficiency type 1
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|a T cell receptor
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| 650 |
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|a Wiskott-Aldrich syndrome
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| 650 |
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|a Cytokines
|2 NLM
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| 650 |
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|a Wiskott-Aldrich Syndrome Protein
|2 NLM
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| 700 |
1 |
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|a Wada, Taizo
|e verfasserin
|4 aut
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| 700 |
1 |
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|a Morio, Tomohiro
|e verfasserin
|4 aut
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| 773 |
0 |
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|i Enthalten in
|t Clinical immunology (Orlando, Fla.)
|d 1999
|g 242(2022) vom: 15. Sept., Seite 109098
|w (DE-627)NLM098196855
|x 1521-7035
|7 nnas
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| 773 |
1 |
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|g volume:242
|g year:2022
|g day:15
|g month:09
|g pages:109098
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| 856 |
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|u http://dx.doi.org/10.1016/j.clim.2022.109098
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