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Statements

Subject Item
n2:RIV%2F00216224%3A14110%2F09%3A00038938%21RIV10-MSM-14110___
rdf:type
skos:Concept n17:Vysledek
dcterms: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.
dcterms: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.
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.
skos:notation
RIV/00216224:14110/09:00038938!RIV10-MSM-14110___
n4:aktivita
n9:S
n4:aktivity
S
n4:cisloPeriodika
4
n4:dodaniDat
n10:2010
n4:domaciTvurceVysledku
n15:9731490
n4:druhVysledku
n12:J
n4:duvernostUdaju
n6:S
n4:entitaPredkladatele
n8:predkladatel
n4:idSjednocenehoVysledku
351992
n4:idVysledku
RIV/00216224:14110/09:00038938
n4:jazykVysledku
n11:eng
n4:klicovaSlova
Wiskott-Aldrich syndrome; NKT cells; intracellular signalling
n4:klicoveSlovo
n5:intracellular%20signalling n5:NKT%20cells n5:Wiskott-Aldrich%20syndrome
n4:kodStatuVydavatele
GB - Spojené království Velké Británie a Severního Irska
n4:kontrolniKodProRIV
[2987EA05AB2F]
n4:nazevZdroje
J.Exp. Med
n4:obor
n14:EC
n4:pocetDomacichTvurcuVysledku
1
n4:pocetTvurcuVysledku
17
n4:rokUplatneniVysledku
n10:2009
n4:svazekPeriodika
206
n4:tvurceVysledku
Villa, Anna Aiuti, Alessandro Casorati, Giulia Draghici, Elena Bredius, Robbert Loci, Michaela Litzman, Jiří Marangoni, Francesco Trasher, Adrian J. Cancrini, Caterina Catucci, Marco Bosticardo, Marita Marodi, Laszlo Espanol, Terasa Roncarolo, Maria Grazia Schulz, Ansgar Dellabona, Paolo
n4:wos
000266009600003
s:issn
0022-1007
s:numberOfPages
8
n13:organizacniJednotka
14110