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Mouse Model of Alagille Syndrome and Mechanisms of Jagged1 Missense Mutations.

Gastroenterology. 2018;

Andersson Emma R，Chivukula Indira V，Hankeova Simona，Sj?qvist Marika，Tsoi Yat Long，Ramsk?ld Daniel，Masek Jan，Elmansuri Aiman，Hoogendoorn Anita，Vazquez Elenae，Storvall Helena，Netu?ilová Julie，Huch Meritxell，Fischler Bj?rn，Ellis Ewa，Contreras Adriana，Nemeth Antal，Chien Kenneth C，Clevers Hans，Sandberg Rickard，Bryja Vitezslav，Lendahl U

Products/Services Used	Details	Operation
Molecular Biology Reagents	ng/well) or human full length Notch2 (50 ng/well, plasmids were either purchased from Genscript	Get A Quote

摘要

Alagille syndrome is a genetic disorder characterized by cholestasis， ocular abnormalities， characteristic facial features， heart defects， and vertebral malformations. Most cases are associated with mutations in JAGGED1 (JAG1)， which encodes a Notch ligand， although it is not clear how these contribute to disease development. We aimed to develop a mouse model of Alagille syndrome to elucidate these mechanisms.