Találati lista | Tudóstér

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
Borító:
Saját polcon: