Inhibition of Jagged-mediated Notch signaling disrupts zebrafish biliary development and generates multi-organ defects compatible with an Alagille syndrome phenocopy
Copyright policy )Inhibition of Jagged-mediated Notch signaling disrupts zebrafish biliary development and generates multi-organ defects compatible with an Alagille syndrome 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.
- National Institute of Health Pakistan
- University of Pennsylvania United States
- Children's Hospital of Philadelphia United States
Mammals, Receptors, Notch, Calcium-Binding Proteins, Membrane Proteins, Zebrafish Proteins, Ligands, Alagille Syndrome, Animals, Genetically Modified, Liver, Microscopy, Electron, Transmission, Animals, Intercellular Signaling Peptides and Proteins, Serrate-Jagged Proteins, Bile Ducts, Jagged-2 Protein, Biliary Tract, Jagged-1 Protein, Zebrafish, Signal Transduction
Mammals, Receptors, Notch, Calcium-Binding Proteins, Membrane Proteins, Zebrafish Proteins, Ligands, Alagille Syndrome, Animals, Genetically Modified, Liver, Microscopy, Electron, Transmission, Animals, Intercellular Signaling Peptides and Proteins, Serrate-Jagged Proteins, Bile Ducts, Jagged-2 Protein, Biliary Tract, Jagged-1 Protein, Zebrafish, Signal Transduction
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