Impaired Toll-like receptor 8–mediated IL-6 and TNF-α production in antigen-presenting cells from patients with X-linked agammaglobulinemia-Reference-Cited by-同舟云学术

Impaired Toll-like receptor 8–mediated IL-6 and TNF-α production in antigen-presenting cells from patients with X-linked agammaglobulinemia

Published:2006-11-07 Issue:6 Volume:109 Page:2553-2556
ISSN:0006-4971
Container-title:Blood
language:en
Short-container-title:

Author:

Sochorová Klára¹,Horváth Rudolf¹,Rožková Daniela¹,Litzman Jiří²,Bartůňková Jiřina¹,Šedivá Anna¹,Špíšek Radek¹

Affiliation:

1. Institute of Immunology, Charles University, 2nd Medical School, Prague, Czech Republic;

2. Department of Clinical Immunology and Allergology, Masaryk University, Brno, Czech Republic

Abstract

Abstract The critical role of Bruton tyrosine kinase (Btk) in B cells has been documented by the block of B-cell development in X-linked agammaglobulinemia (XLA). Less is known about Btk function in myeloid cells. Several pieces of evidence indicate that Btk is a component of Toll-like receptor (TLR) signaling. We analyzed whether Btk deficiency in XLA is associated with an impaired dendritic cell (DC) compartment or defective TLR signaling. We analyzed the expression of TLRs 1 to 9 on myeloid DCs generated from XLA patients and evaluated their response to activation by specific TLR agonists. We show that XLA patients have normal numbers of circulating DCs. Btk-deficient DCs have no defect in response to stimulation of TLRs 1/2, 2/6, 3, 4, and 5 but display a profound impairment of IL-6 and TNF-α production in response to stimulation by TLR-8 cognate agonist, ssRNA. These findings may provide an explanation for the susceptibility to enteroviral infections in XLA patients.

Publisher

American Society of Hematology

Subject

Cell Biology,Hematology,Immunology,Biochemistry

Link

http://ashpublications.org/blood/article-pdf/109/6/2553/1477764/zh800607002553.pdf

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