Protection from liver cancer in a mouse model of Alagille syndrome follows dysregulated differentiation of thymocytes and hepatocytes

• Published in: bioRxiv
• Main Authors: Masek, Jan, Filipovic, Iva, Hankeova, Simona, He, Jingyan, Noemi Km Van Hul, Belicova, Lenka, Jirouskova, Marketa, Frontino, Anna Maria, Turetti, Fabio, Oliveira, Daniel V, Cervenka, Igor, Sarnova, Lenka, Verboven, Elisabeth, Brabec, Tomas, Bjorkstrom, Niklas K, Martin, Gregor, Dobes, Jan, Andersson, Emma R
• Format: Paper
• Language: English
• Published: Cold Spring Harbor Cold Spring Harbor Laboratory Press 25.10.2022
• Subjects: Alagille syndrome; Bile ducts; Carcinogenesis; CD4 antigen; CD8 antigen; Cell activation; Cholestasis; Embryos; Fibrosis; Flow cytometry; Gallbladder diseases; Hepatocytes; Immune system; Inflammatory bowel diseases; Jagged1 protein; Liver cancer; Liver diseases; Lymphocytes T; Pathology; Patients; Pediatrics; RAG1 protein; Thymocytes; Thymus; Tumors; Ulcerative colitis

Background and Aims: Alagille syndrome (ALGS) is a pediatric genetic disorder, caused by mutations in the Notch ligand JAGGED1, presenting with cholestasis due to intrahepatic bile duct paucity. Despite chronic liver disease, few patients develop severe fibrosis or liver cancer, compared to other ch...