Article
Jagged1 in the portal vein mesenchyme regulates intrahepatic bile duct development: insights into Alagille syndrome.
Development (Cambridge, England) - 1 Dec 2010
Hofmann Jennifer J, Zovein Ann C, Koh Huilin, Radtke Freddy, Weinmaster Gerry, Iruela-Arispe M Luisa
Abstract excerpt
Mutations in the human Notch ligand jagged 1 (JAG1) result in a multi-system disorder called Alagille syndrome (AGS). AGS is chiefly characterized by a paucity of intrahepatic bile ducts (IHBD), but also includes cardiac, ocular, skeletal, craniofacial and renal defects. The disease penetration and severity of the affected organs can vary significantly and the molecular basis for this broad spectrum of pathology...
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