Article
JAG1 c.1615C > T mutation impairs bile duct regeneration but not differentiation in hepatic organoids derived from a patient with Alagille syndrome.
Stem cells translational medicine - 18 Sept 2026
Wan Zirui, Zhang Haiyan, Zhang Lu, Cao Xiaofang, An Lisha, Wang Bin, Guan Jingyun, Jia Jinlin, Liu Chen, Jin Xiaohua, Li Yue, Liu Guohua, Ma Xu
Abstract excerpt
OBJECTIVE: Alagille syndrome (ALGS) is an autosomal dominant disorder caused primarily by mutations in JAG1. Intrahepatic bile duct paucity is the most consistently reported feature of ALGS; however, the mechanisms associated with biliary defects remain unclear. METHODS: Here, we reverted or introduced the JAG1 c.1615C > T mutation to patient-specific induced pluripotent stem cells (iPSC) or human embryonic stem...
Read the complete abstract on PubMedTopics
- Humans
- Alagille Syndrome
- Jagged-1 Protein
- Organoids
- Cell Differentiation
- Induced Pluripotent Stem Cells