Journal
DEVELOPMENT
Volume 131, Issue 22, Pages 5753-5766Publisher
COMPANY OF BIOLOGISTS LTD
DOI: 10.1242/dev.01411
Keywords
zebrafish; Notch; jagged; Alagille syndrome; bile duct.; biliary development
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Funding
- NIDDK NIH HHS [DK60369] Funding Source: Medline
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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.
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