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

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  • 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
http://linked.open...avai/riv/aktivity
  • S
http://linked.open...iv/cisloPeriodika
  • 4
http://linked.open...vai/riv/dodaniDat
http://linked.open...aciTvurceVysledku
http://linked.open.../riv/druhVysledku
http://linked.open...iv/duvernostUdaju
http://linked.open...titaPredkladatele
http://linked.open...dnocenehoVysledku
  • 351992
http://linked.open...ai/riv/idVysledku
  • RIV/00216224:14110/09:00038938
http://linked.open...riv/jazykVysledku
http://linked.open.../riv/klicovaSlova
  • Wiskott-Aldrich syndrome; NKT cells; intracellular signalling (en)
http://linked.open.../riv/klicoveSlovo
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
http://linked.open...ichTvurcuVysledku
http://linked.open...cetTvurcuVysledku
http://linked.open...UplatneniVysledku
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
http://localhost/t...ganizacniJednotka
  • 14110
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