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231225s2019 xx |||||o 00| ||eng c |
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|a 10.1016/j.clim.2019.04.012
|2 doi
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|a pubmed25n0988.xml
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|a (NLM)31028919
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|a (PII)S1521-6616(18)30744-7
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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 Posovszky, Carsten
|e verfasserin
|4 aut
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| 245 |
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|a Persisting enteropathy and disturbed adaptive mucosal immunity due to MHC class II deficiency
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|c 2019
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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
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|2 rdacarrier
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|a Date Completed 02.04.2020
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|a Date Revised 02.04.2020
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|a published: Print-Electronic
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|a Citation Status MEDLINE
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|a Copyright © 2019 Elsevier Inc. All rights reserved.
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|a Intestinal epithelial cells (IECs) form a fundamental mucosal barrier and actively participate in tolerance and immunity against intestinal contents. Major histocompatibility complex class II (MHC II) and invariant chain (Ii) molecules are essential for adaptive immune response. MHC II deficiency often presents with gastrointestinal disorders. Intestinal biopsy samples revealed an absence of HLA-DR, Ii, and local immunoglobulins in both hematopoietic immune cells and IECs accompanied by a lack of faecal sIgA. After successful hematopoietic stem cell transplantation (HSCT) absent HLA-DR and Ii expression persisted in IECs and faecal stool analysis indicated inflammation and high microbial activity. We describe multifaceted disturbance of adaptive mucosal immunity in MHC II deficient patients suffering from enteropathy. HLA-DR and Ii expression on enterocytes is not restored by HSCT. This may account for increased susceptibility to enteric infections and intestinal inflammation leading to prolonged enteropathy reported in MHC II deficient patients
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|a Journal Article
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|a Adaptive immunity
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|a Enteropathy
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|a HLA-DR
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|a Hematopoietic stem cell transplantation
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|a Intestinal epithelial cells
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|a Invariant chain
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|a MHC class II deficiency
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|a Mucosal immunity
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|a DNA-Binding Proteins
|2 NLM
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|a HLA-DR Antigens
|2 NLM
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|a RFXANK protein, human
|2 NLM
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|a RFXAP protein, human
|2 NLM
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|a Transcription Factors
|2 NLM
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| 700 |
1 |
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|a Sirin, Mehtap
|e verfasserin
|4 aut
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|a Jacobsen, Eva
|e verfasserin
|4 aut
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| 700 |
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|a Lorenz, Myriam
|e verfasserin
|4 aut
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| 700 |
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|a Schwarz, Klaus
|e verfasserin
|4 aut
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|a Schmidt-Choudhury, Anjona
|e verfasserin
|4 aut
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| 700 |
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|a Rothoeft, Tobias
|e verfasserin
|4 aut
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| 700 |
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|a Schuetz, Catharina
|e verfasserin
|4 aut
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| 700 |
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|a Hönig, Manfred
|e verfasserin
|4 aut
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| 700 |
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|a Debatin, Klaus-Michael
|e verfasserin
|4 aut
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| 700 |
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|a Schulz, Ansgar
|e verfasserin
|4 aut
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| 700 |
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|a Möller, Peter
|e verfasserin
|4 aut
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| 700 |
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|a Barth, Thomas F
|e verfasserin
|4 aut
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| 773 |
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|i Enthalten in
|t Clinical immunology (Orlando, Fla.)
|d 1999
|g 203(2019) vom: 15. Juni, Seite 125-133
|w (DE-627)NLM098196855
|x 1521-7035
|7 nnns
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| 773 |
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|g volume:203
|g year:2019
|g day:15
|g month:06
|g pages:125-133
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| 856 |
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|u http://dx.doi.org/10.1016/j.clim.2019.04.012
|3 Volltext
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