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First published online October 27, 2004
doi: 10.1242/10.1242/dev.01411
1 Department of Medicine, University of Pennsylvania School of Medicine, 421
Curie Boulevard, Philadelphia, PA 19104-6058, USA
2 Laboratory of Molecular Genetics, NICHD, NIH, 31 Center Drive, 9000 Rockville
Pike, Bethesda, MD 20892-2425, USA
3 Genome Technology Branch, NHGRI, NIH, 49 Convent Drive, 9000 Rockville Pike,
Bethesda, MD 20892-2152, USA
4 Division of Gastroenterology and Nutrition, Children's Hospital of
Philadelphia, 34th Street and Civic Center Boulevard, Philadelphia, PA 19104,
USA
5 Department of Cell and Developmental Biology, University of Pennsylvania
School of Medicine, Philadelphia, PA 19104-6058, USA
Author for correspondence (e-mail:
mpack{at}mail.med.upenn.edu)
Accepted 20 August 2004
The Alagille Syndrome (AGS) is a heritable disorder affecting the liver and other organs. Causative dominant mutations in human Jagged 1 have been identified in most AGS patients. Related organ defects occur in mice that carry jagged 1 and notch 2 mutations. Multiple jagged and notch genes are expressed in the developing zebrafish liver. Compound jagged and notch gene knockdowns alter zebrafish biliary, kidney, pancreatic, cardiac and craniofacial development in a manner compatible with an AGS phenocopy. These data confirm an evolutionarily conserved role for Notch signaling in vertebrate liver development, and support the zebrafish as a model system for diseases of the human biliary system.
Key words: Zebrafish, Notch, Jagged, Alagille Syndrome, Bile Duct, Biliary Development
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