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20231225165322.0 |
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231225s2021 xx |||||o 00| ||eng c |
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|a 10.1016/j.clim.2020.108638
|2 doi
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|a pubmed24n1061.xml
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|a (DE-627)NLM318421283
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|a (NLM)33276124
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|a (PII)S1521-6616(20)30798-1
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|a DE-627
|b ger
|c DE-627
|e rakwb
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|a eng
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| 100 |
1 |
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|a Renner, Ellen D
|e verfasserin
|4 aut
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1 |
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|a Class Switch Recombination Defects
|b impact on B cell maturation and antibody responses
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|c 2021
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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 16.06.2021
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|a Date Revised 16.06.2021
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|a published: Print-Electronic
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|a Citation Status MEDLINE
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|a Copyright © 2020 Elsevier Inc. All rights reserved.
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|a To assess how B cell phenotype analysis correlates with antigen responses in patients with class switch recombination defects (CSRD) we quantified memory B cells by flow-cytometry and immunized CSRD patients with the neoantigen bacteriophage phiX174 (phage). CSRD patients showed uniformly absent or markedly reduced switched memory B cells (IgM-IgD-CD27+). CD40L patients had reduced CD27+ memory B cells (both non-switched and switched). In NEMO patients, results varied depending on the IKKγ gene variant. Three of four AID patients had normal percentages of CD27+ memory B cells while CD27+IgM-IgD- switched memory B cells were markedly reduced in all AID patients. Antibody response to phage was remarkably decreased with lack of memory amplification and class-switching in immunized CD40L, UNG deficient, and NEMO patients. Distinct B-cell phenotype pattern correlated with abnormal antibody responses to a T-cell dependent neoantigen, representing a powerful tool to identify CSRD patients
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|a Journal Article
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|a Research Support, Non-U.S. Gov't
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|a B cell development
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|a Class switch recombination defect (CSRD)
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| 650 |
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|a Clinical immunology
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|a Hyper-IgM syndromes (HIGM)
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|a Immunodeficiencies
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| 650 |
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|a I-kappa B Proteins
|2 NLM
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|a Immunoglobulin D
|2 NLM
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|a Immunoglobulin M
|2 NLM
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|a Tumor Necrosis Factor Receptor Superfamily, Member 7
|2 NLM
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| 650 |
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|a CD40 Ligand
|2 NLM
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| 650 |
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|a 147205-72-9
|2 NLM
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| 700 |
1 |
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|a Krätz, Carolin E
|e verfasserin
|4 aut
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| 700 |
1 |
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|a Orange, Jordan S
|e verfasserin
|4 aut
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| 700 |
1 |
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|a Hagl, Beate
|e verfasserin
|4 aut
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| 700 |
1 |
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|a Rylaarsdam, Stacey
|e verfasserin
|4 aut
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| 700 |
1 |
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|a Notheis, Gundula
|e verfasserin
|4 aut
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| 700 |
1 |
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|a Durandy, Anne
|e verfasserin
|4 aut
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| 700 |
1 |
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|a Torgerson, Troy R
|e verfasserin
|4 aut
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| 700 |
1 |
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|a Ochs, Hans D
|e verfasserin
|4 aut
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| 773 |
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|i Enthalten in
|t Clinical immunology (Orlando, Fla.)
|d 1999
|g 222(2021) vom: 15. Jan., Seite 108638
|w (DE-627)NLM098196855
|x 1521-7035
|7 nnns
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| 773 |
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|g volume:222
|g year:2021
|g day:15
|g month:01
|g pages:108638
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| 856 |
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|u http://dx.doi.org/10.1016/j.clim.2020.108638
|3 Volltext
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