Article
Intrahepatic biliary anomalies in a patient with Mowat-Wilson syndrome uncover a role for the zinc finger homeobox gene zfhx1b in vertebrate biliary development.
Journal of pediatric gastroenterology and nutrition - 1 Mar 2011
Cui Shuang, Erlichman Jessi, Russo Pierre, Haber Barbara A, Matthews Randolph P
Abstract excerpt
BACKGROUND: zfhz1b is the causative gene for Mowat-Wilson syndrome, in which patients demonstrate developmental delay and Hirschsprung disease, as well as other anomalies. MATERIALS AND METHODS: We identified a patient with Mowat-Wilson syndrome who also developed cholestasis and histopathologic features consistent with biliary atresia, suggesting that mutations involving zfhz1b may lead to biliary developmental...
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