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Implication of Innate Immunity in the Pathogenesis of Biliary Atresia

Author(s): Jiin-Haur Chuang | Ming-Huei Chou | Chia-Ling Wu | Yung-Ying Du

Journal: Chang Gung Medical Journal
ISSN 2072-0939

Volume: 29;
Issue: 03;
Start page: 240;
Date: 2006;
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Keywords: biliary atresia | innate immunity | CD14 | toll-like receptor.

Biliary atresia (BA) is a complex disorder for which the etiology is still far from clear.Newborn infants that develop BA may carry certain genetic defects, resulting in susceptibilityto uncertain pathogens with characteristic pathogen-associated molecular patterns(PAMPs). The pathogens with their characteristic PAMPs in turn lead to activation of theinnate immune system by triggering pattern recognition receptors on the immune cells. Tolllikereceptors (TLRs) are the most recognized pattern recognition receptors and TLR signalingis the telltale sign of activation of innate immunity. The activation of TLR and the innateimmune system in BA is demonstrated by the up-regulation of TLR7 and by the associationof promoter polymorphism of CD14 with BA. The antimicrobial peptide hepcidin and MxA,a protein downstream of TLR7 signaling, which is also known as a highly specific markerfor type I IFN signaling, are also found highly expressed in the early stage of BA. Thisreview examines the known components of innate immunity involved in BA and outlinesthe potential role of the innate immune system, in cooperation with adaptive immunity, inthe pathogenesis of BA.
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