Alagille syndrome (AGS) is a developmental disorder affecting multiple organs, including the liver, in which bile duct paucity is observed. Clinical presentation varies widely, but nearly all AGS patients have altered Notch signalling. By studying the 3D structure of the liver in conditional mouse strains, Sparks et al. show that altered Notch signalling not only disrupts normal development of bile ducts, as previously reported, but also causes a progressive obstruction of the bile duct network with age. These results provide new clues about the complex mechanisms of liver dysfunction in AGS, and may help to manage AGS patients that present with evolving bile duct obstruction.
- Written by editorial staff. © 2011. Published by The Company of Biologists Ltd.
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