| LOCI, Michaela, Elena DRAGHICI, Francesco MARANGONI, Marita BOSTICARDO, Marco CATUCCI, Alessandro AIUTI, Caterina CANCRINI, Laszlo MARODI, Terasa ESPANOL, Robbert BREDIUS, Adrian J. TRASHER, Ansgar SCHULZ, Jiří LITZMAN, Maria Grazia RONCAROLO, Giulia CASORATI, Anna VILLA a Paolo DELLABONA. The Wiskott-Aldrich syndrome protein is required for iNKT cell maturation and function. J.Exp. Med. 2009, roč. 206, č. 4, s. 735-742. ISSN 0022-1007. |
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@article{871447,
author = {Loci, Michaela and Draghici, Elena and Marangoni, Francesco and Bosticardo, Marita and Catucci, Marco and Aiuti, Alessandro and Cancrini, Caterina and Marodi, Laszlo and Espanol, Terasa and Bredius, Robbert and Trasher, Adrian J. and Schulz, Ansgar and Litzman, Jiří and Roncarolo, Maria Grazia and Casorati, Giulia and Villa, Anna and Dellabona, Paolo},
article_number = {4},
keywords = {Wiskott-Aldrich syndrome; NKT cells; intracellular signalling},
language = {eng},
issn = {0022-1007},
journal = {J.Exp. Med},
title = {The Wiskott-Aldrich syndrome protein is required for iNKT cell maturation and function.},
volume = {206},
year = {2009}
}
TY - JOUR
ID - 871447
AU - Loci, Michaela - Draghici, Elena - Marangoni, Francesco - Bosticardo, Marita - Catucci, Marco - Aiuti, Alessandro - Cancrini, Caterina - Marodi, Laszlo - Espanol, Terasa - Bredius, Robbert - Trasher, Adrian J. - Schulz, Ansgar - Litzman, Jiří - Roncarolo, Maria Grazia - Casorati, Giulia - Villa, Anna - Dellabona, Paolo
PY - 2009
TI - The Wiskott-Aldrich syndrome protein is required for iNKT cell maturation and function.
JF - J.Exp. Med
VL - 206
IS - 4
SP - 735-742
EP - 735-742
SN - 00221007
KW - Wiskott-Aldrich syndrome
KW - NKT cells
KW - intracellular signalling
N2 - 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. was(-/-) iNKT cells were also functionally impaired, as suggested by the reduced secretion of interleukin 4 and interferon gamma upon in vivo activation. Altogether, these results demonstrate the relevance of WASp in integrating signals critical for development and functional differentiation of iNKT cells and suggest that defects in these cells may play a role in WAS pathology
ER -
LOCI, Michaela, Elena DRAGHICI, Francesco MARANGONI, Marita BOSTICARDO, Marco CATUCCI, Alessandro AIUTI, Caterina CANCRINI, Laszlo MARODI, Terasa ESPANOL, Robbert BREDIUS, Adrian J. TRASHER, Ansgar SCHULZ, Jiří LITZMAN, Maria Grazia RONCAROLO, Giulia CASORATI, Anna VILLA a Paolo DELLABONA. The Wiskott-Aldrich syndrome protein is required for iNKT cell maturation and function. \textit{J.Exp. Med}. 2009, roč.~206, č.~4, s.~735-742. ISSN~0022-1007.
|
