Article
Premature senescence of the liver in Alagille patients.
PloS one - 1 Jan 2023
Jannone Giulia, de Magnée Catherine, Tambucci Roberto, Evraerts Jonathan, Ravau Joachim, Najimi Mustapha, Sokal Etienne Marc
Abstract excerpt
INTRODUCTION: Alagille syndrome (ALGS) is an autosomal dominant disease characterized by a multisystem involvement including bile duct paucity and cholestasis, caused by JAG1 or NOTCH2 mutations in most of the cases. Jagged1-Notch2 interactions are known to be crucial for intrahepatic biliary tract development, but the Notch signaling pathway is also involved in the juxtacrine transmission of senescence and in...
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