DNA hypomethylation causes bile duct defects in zebrafish and is a distinguishing feature of infantile biliary atresia.

ABSTRACT:

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Infantile cholestatic disorders arise in the context of progressively developing intrahepatic bile ducts. Biliary atresia (BA), a progressive fibroinflammatory disorder of extra- and intrahepatic bile ducts, is the most common identifiable cause of infantile cholestasis and the leading indication for liver transplantation in children. The etiology of BA is unclear, and although there is some evidence for viral, toxic, and complex genetic causes, the exclusive occurrence of BA during a period of biliary growth and remodeling suggests an importance of developmental factors. Interestingly, interferon-γ (IFN-γ) signaling is activated in patients and in the frequently utilized rhesus rotavirus mouse model of BA, and is thought to play a key mechanistic role. Here we demonstra

SUBMITTER: Matthews RP 

PROVIDER: S-EPMC3075951 | biostudies-literature | 2011 Mar

REPOSITORIES: biostudies-literature