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

J 2009

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

LOCI, Michaela, Elena DRAGHICI, Francesco MARANGONI, Marita BOSTICARDO, Marco CATUCCI et. al.

Základní údaje

Originální název

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

Název česky

WASP ne nutný pro maturaci a funkci iNKT buněk

Autoři

LOCI, Michaela (380 Itálie), Elena DRAGHICI (380 Itálie), Francesco MARANGONI (372 Irsko), Marita BOSTICARDO (380 Itálie), Marco CATUCCI (380 Itálie), Alessandro AIUTI (380 Itálie), Caterina CANCRINI (380 Itálie), Laszlo MARODI (348 Maďarsko), Terasa ESPANOL (724 Španělsko), Robbert BREDIUS (208 Dánsko), Adrian J. TRASHER (826 Velká Británie a Severní Irsko), Ansgar SCHULZ (276 Německo), Jiří LITZMAN (203 Česká republika, garant), Maria Grazia RONCAROLO (380 Itálie), Giulia CASORATI (380 Itálie), Anna VILLA (380 Itálie) a Paolo DELLABONA (380 Itálie)

Vydání

J.Exp. Med, 2009, 0022-1007

Další údaje

Jazyk

angličtina

Typ výsledku

Článek v odborném periodiku

Obor

30102 Immunology

Stát vydavatele

Velká Británie a Severní Irsko

Utajení

není předmětem státního či obchodního tajemství

Impakt faktor

Impact factor: 14.505

Kód RIV

RIV/00216224:14110/09:00038938

Organizační jednotka

Lékařská fakulta

UT WoS

000266009600003

Klíčová slova česky

Wiskotův -Aldrichův syndrom; KNT buňky; ingtracelulární signalizace

Klíčová slova anglicky

Wiskott-Aldrich syndrome; NKT cells; intracellular signalling

Příznaky

Mezinárodní význam, Recenzováno

Změněno: 2. 4. 2010 07:58, prof. MUDr. Jiří Litzman, CSc.

Anotace

ORIG CZ

V originále

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

Česky

I nemocných s Wiskottovým-aldrichovým syndrerom bylo nalezení snížení počtu iNKT buněk v periferní krvi. Byla zkonstruována wasp-/- myš, u ní byl počet iNKT buněk nižší než u kontrolních myší. Výsledky ukazují na výzman WASP při intracelulární signalizaci při vzniku iNKT buněk.

Citovat

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.

@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.

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