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Title: Mutations in STAT3 and IL12RB1 impair the development of human IL-17-producing T cells
Authors: De Beaucoudtey, Ludovic
Puel, Anne
Filipe-Santos, Orchidée
Cobat, Aurélie
Ghandil, Pegah
Chrabieh, Maya
Felnberg, Jacqueline
Von Bernuth, Horst
Samarina, Arina
Jannière, Lucile
Fieschi, Claire
Stéphan, Jean Louis
Boileau, Catherine
Lyonnet, Stanislas
Jondeau, Guillaume
Cormier-Daire, Valérie
Le Merrer, Martine
Hoarau, Cyrille
Lebranchu, Yvon
Lortholary, Olivier
Chandesris, Marie Olivia
Tron, François
Gambineri, Eleonora
Bianchi, Lucia
Rodriguez-Gallego, Carlos 
Zitnik, Simona E.
Vasconcelos, Julia
Guedes, Margarida
Vitor, Artur Bonito
Marodi, Laszlo
Chapel, Helen
Reid, Brenda
Roifman, Chaim
Nadal, David
Reichenbach, Janine
Caragol, Isabel
Garty, Ben Zion
Dogu, Figen
Camcioglu, Yildiz
Gülle, Sanyie
Sanal, Ozden
Fischer, Alain
Abel, Laurent
Stockinger, Birgitta
Picard, Capucine
Casanova, Jean Laurent
UNESCO Clasification: 32 Ciencias médicas
3205 Medicina interna
Keywords: STAT3
T cells
Issue Date: 2008
Journal: Journal of Experimental Medicine 
Abstract: The cytokines controlling the development of human interleukin (IL) 17--producing T helper cells in vitro have been difficult to identify. We addressed the question of the development of human IL-17--producing T helper cells in vivo by quantifying the production and secretion of IL-17 by fresh T cells ex vivo, and by T cell blasts expanded in vitro from patients with particular genetic traits affecting transforming growth factor (TGF) beta, IL-1, IL-6, or IL-23 responses. Activating mutations in TGFB1, TGFBR1, and TGFBR2 (Camurati-Engelmann disease and Marfan-like syndromes) and loss-of-function mutations in IRAK4 and MYD88 (Mendelian predisposition to pyogenic bacterial infections) had no detectable impact. In contrast, dominant-negative mutations in STAT3 (autosomal-dominant hyperimmunoglobulin E syndrome) and, to a lesser extent, null mutations in IL12B and IL12RB1 (Mendelian susceptibility to mycobacterial diseases) impaired the development of IL-17--producing T cells. These data suggest that IL-12Rbeta1- and STAT-3--dependent signals play a key role in the differentiation and/or expansion of human IL-17-producing T cell populations in vivo.
ISSN: 0022-1007
DOI: 10.1084/jem.20080321
Source: Journal of Experimental Medicine[ISSN 0022-1007],v. 205, p. 1543-1550 (Julio 2008)
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