Abstract

Biliary atresia (BA) is a progressive obliterative extrahepatic cholangiopathy of infants. Despite successful surgical drainage of bile, most children with BA experience progression of cirrhosis and liver failure. There is evidence that trans-differentiation of epithelial cells into collagen-producing mesenchymal cells during injury occurs secondary to Fibroblast Growth Factor (FGF) and Transforming Growth Factor-β (TGFβ) signaling activation. Therefore, we hypothesized that TGFβ-mediated epithelial-mesenchymal trans-differentiation (EMT) of hepatic progenitor cells contributes to periportal fibrosis associated with BA.

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