|
|
|
|
| LEADER |
01000naa a22002652 4500 |
| 001 |
NLM338094784 |
| 003 |
DE-627 |
| 005 |
20231225235908.0 |
| 007 |
cr uuu---uuuuu |
| 008 |
231225s2022 xx |||||o 00| ||eng c |
| 024 |
7 |
|
|a 10.1016/j.clim.2022.108974
|2 doi
|
| 028 |
5 |
2 |
|a pubmed24n1126.xml
|
| 035 |
|
|
|a (DE-627)NLM338094784
|
| 035 |
|
|
|a (NLM)35278713
|
| 035 |
|
|
|a (PII)S1521-6616(22)00055-9
|
| 040 |
|
|
|a DE-627
|b ger
|c DE-627
|e rakwb
|
| 041 |
|
|
|a eng
|
| 100 |
1 |
|
|a Patrizi, Ornella
|e verfasserin
|4 aut
|
| 245 |
1 |
0 |
|a Lack of DOCK8 impairs the primary biologic functions of human NK cells and abrogates CCR7 surface expression in a WASP-independent manner
|
| 264 |
|
1 |
|c 2022
|
| 336 |
|
|
|a Text
|b txt
|2 rdacontent
|
| 337 |
|
|
|a ƒaComputermedien
|b c
|2 rdamedia
|
| 338 |
|
|
|a ƒa Online-Ressource
|b cr
|2 rdacarrier
|
| 500 |
|
|
|a Date Completed 18.04.2022
|
| 500 |
|
|
|a Date Revised 31.05.2022
|
| 500 |
|
|
|a published: Print-Electronic
|
| 500 |
|
|
|a Citation Status MEDLINE
|
| 520 |
|
|
|a Copyright © 2022 Elsevier Inc. All rights reserved.
|
| 520 |
|
|
|a Dedicator of Cytokinesis 8 (DOCK8) deficiency is a rare form of autosomal recessive combined immunodeficiency. The effect of DOCK8 deficiency on Natural Killer cell biology has not been fully elucidated yet. Thus, we undertook a detailed phenotypic and functional evaluation of NK cells from seven patients with DOCK8 deficiency. Patients' immature CD56bright NK cells were defective in IFN-γ secretion, while their mature CD56dim NK cells showed impaired cytotoxicity, partially rescued upon rIL-2 addition. Cross-linking of NK cell receptors revealed a specific defect in the CD3 zeta chain-dependent activation pathway in DOCK8 deficiency. Lack of DOCK8, but not of WASP, impaired CCR7 expression on human CD56bright NK cells, a critical receptor for their migration to secondary lymph nodes. Evaluation of a patient's lymph node showed a severe reduction in NK cells that showed increased intracellular expression of CCR7. Our data suggest that DOCK8 deficiency variably affects NK cell homeostasis in humans
|
| 650 |
|
4 |
|a Journal Article
|
| 650 |
|
4 |
|a C-C motif chemokine receptor 7
|
| 650 |
|
4 |
|a Dedicator of cytokinesis 8
|
| 650 |
|
4 |
|a Natural killer cells
|
| 650 |
|
4 |
|a Wiskott-Aldrich syndrome protein
|
| 650 |
|
7 |
|a CCR7 protein, human
|2 NLM
|
| 650 |
|
7 |
|a CD56 Antigen
|2 NLM
|
| 650 |
|
7 |
|a DOCK8 protein, human
|2 NLM
|
| 650 |
|
7 |
|a Guanine Nucleotide Exchange Factors
|2 NLM
|
| 650 |
|
7 |
|a Receptors, CCR7
|2 NLM
|
| 650 |
|
7 |
|a WAS protein, human
|2 NLM
|
| 650 |
|
7 |
|a Wiskott-Aldrich Syndrome Protein
|2 NLM
|
| 700 |
1 |
|
|a Baronio, Manuela
|e verfasserin
|4 aut
|
| 700 |
1 |
|
|a Gazzurelli, Luisa
|e verfasserin
|4 aut
|
| 700 |
1 |
|
|a Rossi, Stefano
|e verfasserin
|4 aut
|
| 700 |
1 |
|
|a Rezzola, Sara
|e verfasserin
|4 aut
|
| 700 |
1 |
|
|a Marcenaro, Emanuela
|e verfasserin
|4 aut
|
| 700 |
1 |
|
|a Plebani, Alessandro
|e verfasserin
|4 aut
|
| 700 |
1 |
|
|a Badolato, Raffaele
|e verfasserin
|4 aut
|
| 700 |
1 |
|
|a Parolini, Silvia
|e verfasserin
|4 aut
|
| 700 |
1 |
|
|a Lougaris, Vassilios
|e verfasserin
|4 aut
|
| 700 |
1 |
|
|a Tabellini, Giovanna
|e verfasserin
|4 aut
|
| 773 |
0 |
8 |
|i Enthalten in
|t Clinical immunology (Orlando, Fla.)
|d 1999
|g 237(2022) vom: 04. Apr., Seite 108974
|w (DE-627)NLM098196855
|x 1521-7035
|7 nnns
|
| 773 |
1 |
8 |
|g volume:237
|g year:2022
|g day:04
|g month:04
|g pages:108974
|
| 856 |
4 |
0 |
|u http://dx.doi.org/10.1016/j.clim.2022.108974
|3 Volltext
|
| 912 |
|
|
|a GBV_USEFLAG_A
|
| 912 |
|
|
|a SYSFLAG_A
|
| 912 |
|
|
|a GBV_NLM
|
| 912 |
|
|
|a GBV_ILN_11
|
| 912 |
|
|
|a GBV_ILN_24
|
| 912 |
|
|
|a GBV_ILN_350
|
| 951 |
|
|
|a AR
|
| 952 |
|
|
|d 237
|j 2022
|b 04
|c 04
|h 108974
|