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001-es BibID:	BIBFORM005160
Első szerző:	de Beaucoudrey, Ludovic
Cím:	Mutations in STAT3 and IL12RB1 impair the development of human IL-17 producing T cells / Ludovic de Beaucoudrey, Anne Puel, Orchidée Filipe-Santos, Aurélie Cobat, Pegah Ghandil, Maya Chrabieh, Jacqueline Feinberg, Horst von Bernuth, Arina Samarina, Lucile Janniere, Claire Fieschi, Jean-Louis Stéphan, Catherine Boileau, Stanislas Lyonnet, Guillaume Jondeau, Valérie Cormier-Daire, Martine Le Merrer, Cyrille Hoarau, Yvon Lebranchu, Olivier Lortholary, Marie-Olivia Chandesris, Francois Tron, Eleonora Gambineri, Lucia Bianchi, Carlos Rodriguez-Gallego, Simona E. Zitnik, Julia Vasconcelos, Margarida Guedes, Artur Bonito Vitor, Laszlo Marodi, Helen Chapel, Brenda Reid, Chaim Roifman, David Nadal, Janine Reichenbach, Isabel Caragol, Ben-Zion Garty, Figen Dogu, Yildiz Camcioglu, Sanyie Gülle, Ozden Sanal, Alain Fischer, Laurent Abel, Birgitta Stockinger, Capucine Picard, and Jean-Laurent Casanova
Dátum:	2008
Tárgyszavak:	Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
Megjelenés:	The Journal of Experimental Medicine. - 205 : 7 (2008), p. 1543-1550. -
További szerzők:	Puel, Anne Filipe-Santos, Orchidée Cobat, Aurélie Ghandil, Pegah Chrabieh, Maya Feinberg, Jacqueline Bernuth, Horst von Samarina, Arina Janniere, 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 Eva Vasconcelos, Julia Guedes, Margarida Bonito Vitor, Artur Maródi László (1949-) (gyermekgyógyász infektológus, immunológus) Chapel, Helen Reid, Brenda Roifman, Chaim Nadal, David Reichenbach, Jeanine Caragol, Isabel Garty, Ben-Zion Dogu, Figen Camcioglu, Yildiz Gülle, Sanyie Sanal, Özden Fischer, Alain Abel, Laurent Stockinger, Birgitta Picard, Capucine Casanova, Jean-Laurent
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001-es BibID:	BIBFORM013546
Első szerző:	Picard, Capucine
Cím:	Clinical features and outcome of patients with IRAK-4 and MyD88 deficiency / Capucine Picard, Horst von Bernuth, Pegah Ghandil, Maya Chrabieh,Ofer Levy, Peter D. Arkwright, Douglas McDonald, Raif S. Geha,Hidetoshi Takada, Jens C. Krause, C. Buddy Creech, Cheng-Lung Ku, Stephan Ehl, László Maródi, Saleh Al-Muhsen, Sami Al-Hajjar, Abdulaziz Al-Ghonaium, Noorbibi K. Day-Good, Steven M. Holland, John I. Gallin,Helen Chapel, David P. Speert, Carlos Rodriguez-Gallego, Elena Colino, Ben-Zion Garty, Chaim Roifman, Toshiro Hara, Hideto Yoshikawa, Shigeaki Nonoyama, Joseph Domachowske, Andrew C. Issekutz, Mimi Tang, Joanne Smart, Simona Eva Zitnik, Cyrille Hoarau, Dinakantha S. Kumararatne, Adrian J. Thrasher, E. Graham Davies, Claire Bethune, Nicolas Sirvent, Dominique de Ricaud, Yildiz Camcioglu, Júlia Vasconcelos, Margarida Guedes, Artur Bonito Vitor, Carlos Rodrigo, Francisco Almazán, Maria Méndez, Juan Ignacio Aróstegui, Laia Alsina, Claudia Fortuny, Janine Reichenbach, James W. Verbsky, Xavier Bossuyt, Rainer Doffinger, Laurent Abel, Anne Puel, and Jean-Laurent Casanova
Dátum:	2010
ISSN:	0025-7974
Tárgyszavak:	Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
Megjelenés:	Medicine. - 89 : 6 (2010), p. 403-425. -
További szerzők:	Bernuth, Horst von Ghandil, Pegah Chrabieh, Maya Levy, Ofer Arkwright, Peter D. McDonald, Douglas Geha, Raif S. Takada, Hidetoshi Krause, Jens Creech, C. Buddy Ku, Cheng-Lung Ehl, Stephan Maródi László (1949-) (gyermekgyógyász infektológus, immunológus) Al-Muhsen, Saleh Al-Hajjar, Sami Al-Ghonaium, Abdulaziz Day-Good, Noorbibi K. Holland, Steven M. Gallin, John Chapel, Helen Speert, David Rodriguez-Gallego, Carlos Colino, Elena Garty, Ben-Zion Roifman, Chaim Hara, Toshiro Yoshikawa, Hideto Nonoyama, Shigeaki Domachowske, Joseph Issekutz, Andrew C. Tang, Mimi Smart, Joanne Zitnik, Simona Eva Hoarau, Cyrille Kumararatne, Dinakantha S. Thrasher, Adrian J. Davies, Graham Bethune, Claire Sirvent, Nicolas de Ricaud, Dominique Camcioglu, Yildiz Vasconcelos, Julia Guedes, Margarida Bonito Vitor, Artur Rodrigo, Carlos Almazán, Francisco Méndez, Maria Aróstegui, Juan Ignacio Alsina, Laia Fortuny, Claudia Reichenbach, Jeanine Verbsky, James W. Bossuyt, Xavier Doffinger, Rainer Abel, Laurent Puel, Anne Casanova, Jean-Laurent
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001-es BibID:	BIBFORM041850
Első szerző:	Weller, Sandra
Cím:	IgM+IgD+CD27+ B cells are markedly reduced in IRAK-4-, MyD88-, and TIRAP- but not UNC-93B-deficient patients / Sandra Weller, Mélanie Bonnet, Héloise Delagreverie, Laura Israel, Maya Chrabieh, László Maródi, Carlos Rodriguez-Gallego, Ben-Zion Garty, Chaim Roifman, Andrew C. Issekutz, Simona Eva Zitnik, Cyrille Hoarau, Yildiz Camcioglu, Júlia Vasconcelos, Carlos Rodrigo, Peter D. Arkwright, Andrea Cerutti, Eric Meffre, Shen-Ying Zhang, Alexandre Alcais, Anne Puel, Jean-Laurent Casanova, Capucine Picard, Jean-Claude Weill, Claude-Agnes Reynaud
Dátum:	2012
ISSN:	0006-4971
Megjegyzések:	We studied the distribution of peripheral B-cell subsets in patients deficient for key factors of the TLR-signaling pathways (MyD88, TIRAP/MAL, IL-1 receptor-associated kinase 4 [IRAK-4], TLR3, UNC-93B, TRIF). All TLRs, except TLR3, which signals through the TRIF adaptor, require MyD88 and IRAK-4 to mediate their function. TLR4 and the TLR2 heterodimers (with TLR1, TLR6, and possibly TLR10) require in addition the adaptor TIRAP, whereas UNC-93B is needed for the proper localization of intracellular TLR3, TLR7, TLR8, and TLR9. We found that IgM(+)IgD(+)CD27(+) but not switched B cells were strongly reduced in MyD88-, IRAK-4-, and TIRAP-deficient patients. This defect did not appear to be compensated with age. However, somatic hypermutation of Ig genes and heavy-chain CDR3 size distribution of IgM(+)IgD(+)CD27(+) B cells were not affected in these patients. In contrast, the numbers of IgM(+)IgD(+)CD27(+) B cells were normal in the absence of TLR3, TRIF, and UNC-93B, suggesting that UNC-93B-dependent TLRs, and notably TLR9, are dispensable for the presence of this subset in peripheral blood. Interestingly, TLR10 was found to be expressed at greater levels in IgM(+)IgD(+)CD27(+) compared with switched B cells in healthy patients. Hence, we propose a role for TIRAP-dependent TLRs, possibly TLR10 in particular, in the development and/or maintenance of IgM(+)IgD(+)CD27(+) B cells in humans.
Tárgyszavak:	Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban külföldön készült közlemény
Megjelenés:	Blood. - 120 : 25 (2012), p. 4992-5001. -
További szerzők:	Bonnet, Mélanie Delagreverie, Héloise Israel, Laura Chrabieh, Maya Rodriguez-Gallego, Carlos Garty, Ben-Zion Roifman, Chaim Issekutz, Andrew C. Zitnik, Simona Eva Hoarau, Cyrille Camcioglu, Yildiz Vasconcelos, Julia Rodrigo, Carlos Arkwright, Peter D. Cerutti, Andrea Meffre, Eric Zhang, Shen-Ying Alcais, Alexandre Puel, Anne Casanova, Jean-Laurent Picard, Capucine Weill, Jean-Claude Reynaud, Claude-Ágnes Maródi László (1949-) (gyermekgyógyász infektológus, immunológus)
Internet cím:	Intézményi repozitóriumban (DEA) tárolt változat DOI
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