Ontology highlight
ABSTRACT:
SUBMITTER: Smith Q
PROVIDER: S-EPMC8787431 | biostudies-literature | 2022 Jan
REPOSITORIES: biostudies-literature
Smith Quinton Q Bays Jennifer J Li Linqing L Shareef Haniyah H Chen Christopher S CS Bhatia Sangeeta N SN
Advanced science (Weinheim, Baden-Wurttemberg, Germany) 20211116 3
Patients with Alagille syndrome carry monogenic mutations in the Notch signaling pathway and face complications such as jaundice and cholestasis. Given the presence of intrahepatic ductopenia in these patients, Notch2 receptor signaling is implicated in driving normal biliary development and downstream branching morphogenesis. As a result, in vitro model systems of liver epithelium are needed to further mechanistic insight of biliary tissue assembly. Here, primary human intrahepatic cholangiocyt ...[more]