About: The Wiskott-Aldrich syndrome protein is required for iNKT cell maturation and function.

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rdf:type	skos:Concept http://linked.opendata.cz/ontology/domain/vavai/Vysledek
Description	The Wiskott-Aldrich syndrome (WAS) protein (WASp) is a regulator of actin cytoskeleton in hematopoietic cells. Mutations of the WASp gene cause WAS. Although WASp is involved in various immune cell functions, its role in invariant natural killer T (iNKT) cells has never been investigated. Defects of iNKT cells could indeed contribute to several WAS features, such as recurrent infections and high tumor incidence. We found a profound reduction of circulating iNKT cells in WAS patients, directly correlating with the severity of clinical phenotype. To better characterize iNKT cell defect in the absence of WASp, we analyzed was(-/-) mice. iNKT cell numbers were significantly reduced in the thymus and periphery of was(-/-) mice as compared with wild-type controls. Moreover analysis of was(-/-) iNKT cell maturation revealed a complete arrest at the CD44(+) NK1.1(-) intermediate stage. Notably, generation of BM chimeras demonstrated a was(-/-) iNKT cell-autonomous developmental defect. The Wiskott-Aldrich syndrome (WAS) protein (WASp) is a regulator of actin cytoskeleton in hematopoietic cells. Mutations of the WASp gene cause WAS. Although WASp is involved in various immune cell functions, its role in invariant natural killer T (iNKT) cells has never been investigated. Defects of iNKT cells could indeed contribute to several WAS features, such as recurrent infections and high tumor incidence. We found a profound reduction of circulating iNKT cells in WAS patients, directly correlating with the severity of clinical phenotype. To better characterize iNKT cell defect in the absence of WASp, we analyzed was(-/-) mice. iNKT cell numbers were significantly reduced in the thymus and periphery of was(-/-) mice as compared with wild-type controls. Moreover analysis of was(-/-) iNKT cell maturation revealed a complete arrest at the CD44(+) NK1.1(-) intermediate stage. Notably, generation of BM chimeras demonstrated a was(-/-) iNKT cell-autonomous developmental defect. (en)
Title	The Wiskott-Aldrich syndrome protein is required for iNKT cell maturation and function. The Wiskott-Aldrich syndrome protein is required for iNKT cell maturation and function. (en)
skos:prefLabel	The Wiskott-Aldrich syndrome protein is required for iNKT cell maturation and function. The Wiskott-Aldrich syndrome protein is required for iNKT cell maturation and function. (en)
skos:notation	RIV/00216224:14110/09:00038938!RIV10-MSM-14110___
http://linked.open...avai/riv/aktivita	S
http://linked.open...avai/riv/aktivity	S
http://linked.open...iv/cisloPeriodika	4
http://linked.open...vai/riv/dodaniDat	2010
http://linked.open...aciTvurceVysledku	Litzman, Jiří
http://linked.open.../riv/druhVysledku	J - Článek v odborném periodiku
http://linked.open...iv/duvernostUdaju	S - Úplné a pravdivé údaje nepodléhající ochraně podle zvláštních právních předpisů
http://linked.open...titaPredkladatele	Masarykova univerzita / Lékařská fakulta
http://linked.open...dnocenehoVysledku	351992
http://linked.open...ai/riv/idVysledku	RIV/00216224:14110/09:00038938
http://linked.open...riv/jazykVysledku	eng - angličtina
http://linked.open.../riv/klicovaSlova	Wiskott-Aldrich syndrome; NKT cells; intracellular signalling (en)
http://linked.open.../riv/klicoveSlovo	Wiskott-Aldrich syndrome intracellular signalling NKT cells
http://linked.open...odStatuVydavatele	GB - Spojené království Velké Británie a Severního Irska
http://linked.open...ontrolniKodProRIV	[2987EA05AB2F]
http://linked.open...i/riv/nazevZdroje	J.Exp. Med
http://linked.open...in/vavai/riv/obor	EC
http://linked.open...ichTvurcuVysledku	1 (xsd:int)
http://linked.open...cetTvurcuVysledku	17 (xsd:int)
http://linked.open...UplatneniVysledku	2009
http://linked.open...v/svazekPeriodika	206
http://linked.open...iv/tvurceVysledku	Litzman, Jiří Aiuti, Alessandro Bosticardo, Marita Bredius, Robbert Cancrini, Caterina Casorati, Giulia Catucci, Marco Dellabona, Paolo Draghici, Elena Espanol, Terasa Loci, Michaela Marangoni, Francesco Marodi, Laszlo Roncarolo, Maria Grazia Schulz, Ansgar Trasher, Adrian J. Villa, Anna
http://linked.open...ain/vavai/riv/wos	000266009600003
issn	0022-1007
number of pages	8 (xsd:int)
http://localhost/t...ganizacniJednotka	14110
is http://linked.open...avai/riv/vysledek of	The Wiskott-Aldrich syndrome protein is required for iNKT cell maturation and function. The Wiskott-Aldrich syndrome protein is required for iNKT cell maturation and function. The Wiskott-Aldrich syndrome protein is required for iNKT cell maturation and function. The Wiskott-Aldrich syndrome protein is required for iNKT cell maturation and function.

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