|
|
|
|
| LEADER |
01000caa a22002652 4500 |
| 001 |
NLM170386007 |
| 003 |
DE-627 |
| 005 |
20240510231947.0 |
| 007 |
tu |
| 008 |
231223s2007 xx ||||| 00| ||eng c |
| 028 |
5 |
2 |
|a pubmed24n1403.xml
|
| 035 |
|
|
|a (DE-627)NLM170386007
|
| 035 |
|
|
|a (NLM)17512803
|
| 040 |
|
|
|a DE-627
|b ger
|c DE-627
|e rakwb
|
| 041 |
|
|
|a eng
|
| 100 |
1 |
|
|a Adriani, Marsilio
|e verfasserin
|4 aut
|
| 245 |
1 |
0 |
|a Impaired in vitro regulatory T cell function associated with Wiskott-Aldrich syndrome
|
| 264 |
|
1 |
|c 2007
|
| 336 |
|
|
|a Text
|b txt
|2 rdacontent
|
| 337 |
|
|
|a ohne Hilfsmittel zu benutzen
|b n
|2 rdamedia
|
| 338 |
|
|
|a Band
|b nc
|2 rdacarrier
|
| 500 |
|
|
|a Date Completed 10.08.2007
|
| 500 |
|
|
|a Date Revised 10.05.2024
|
| 500 |
|
|
|a published: Print-Electronic
|
| 500 |
|
|
|a Citation Status MEDLINE
|
| 520 |
|
|
|a Wiskott-Aldrich syndrome (WAS) is a primary immunodeficiency characterized by the contradictory coexistence of impaired T-cell function and exaggerated T-cell-mediated pathology, including autoimmunity and eczema. WAS protein (WASp)-deficient mice are also immunodeficient and can develop autoimmune disease. Since defects in regulatory T-cells (Treg) are associated with autoimmunity, we examined the presence and function of these cells in WAS patients and WASp-deficient mice. We found that CD4(+)CD25(+)FOXP3(+) Treg cells can develop in the absence of WASp expression. However, Treg cells both from WASp-deficient mice and from four out of five WAS patients studied showed impaired in vitro suppressor function. In WASp-deficient mice, this defect could be partially rescued by pre-activation with IL-2, suggesting that inadequate cell activation may play a role in WASp-deficient Treg dysfunction. These findings may provide insights into the complex pathophysiology and paradoxical phenotypes of WAS and suggest new therapeutic modalities for autoimmunity in these patients
|
| 650 |
|
4 |
|a Journal Article
|
| 650 |
|
4 |
|a Research Support, N.I.H., Intramural
|
| 650 |
|
7 |
|a Forkhead Transcription Factors
|2 NLM
|
| 650 |
|
7 |
|a Interleukin-2
|2 NLM
|
| 650 |
|
7 |
|a Receptors, Interleukin-2
|2 NLM
|
| 650 |
|
7 |
|a Wiskott-Aldrich Syndrome Protein
|2 NLM
|
| 700 |
1 |
|
|a Aoki, Joseph
|e verfasserin
|4 aut
|
| 700 |
1 |
|
|a Horai, Reiko
|e verfasserin
|4 aut
|
| 700 |
1 |
|
|a Thornton, Angela M
|e verfasserin
|4 aut
|
| 700 |
1 |
|
|a Konno, Akihiro
|e verfasserin
|4 aut
|
| 700 |
1 |
|
|a Kirby, Martha
|e verfasserin
|4 aut
|
| 700 |
1 |
|
|a Anderson, Stacie M
|e verfasserin
|4 aut
|
| 700 |
1 |
|
|a Siegel, Richard M
|e verfasserin
|4 aut
|
| 700 |
1 |
|
|a Candotti, Fabio
|e verfasserin
|4 aut
|
| 700 |
1 |
|
|a Schwartzberg, Pamela L
|e verfasserin
|4 aut
|
| 773 |
0 |
8 |
|i Enthalten in
|t Clinical immunology (Orlando, Fla.)
|d 1999
|g 124(2007), 1 vom: 15. Juli, Seite 41-8
|w (DE-627)NLM098196855
|x 1521-7035
|7 nnns
|
| 773 |
1 |
8 |
|g volume:124
|g year:2007
|g number:1
|g day:15
|g month:07
|g pages:41-8
|
| 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 124
|j 2007
|e 1
|b 15
|c 07
|h 41-8
|