Inhibition of Jagged-mediated Notch signaling disrupts zebrafish biliary development and generates multi-organ defects compatible with an Alagille syndrome …
K Lorent, SY Yeo, T Oda, S Chandrasekharappa… - 2004 - journals.biologists.com
K Lorent, SY Yeo, T Oda, S Chandrasekharappa, A Chitnis, RP Matthews, M Pack
2004•journals.biologists.comThe 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 …
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 …
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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