Liver‐specific DICER1 syndrome model mice develop cystic liver tumors with defective primary cilia

• Published in: The Journal of pathology Vol. 264; no. 1; pp. 17 - 29
• Main Authors: Oikawa, Keiki, Ohno, Shin‐ichiro, Ono, Kana, Hirao, Kaito, Murakami, Ayano, Harada, Yuichirou, Kumagai, Katsuyoshi, Sudo, Katsuko, Takanashi, Masakatsu, Ishikawa, Akio, Mineo, Shouichirou, Fujita, Koji, Umezu, Tomohiro, Watanabe, Noriko, Murakami, Yoshiki, Ogawa, Shinichiro, Schultz, Kris Ann, Kuroda, Masahiko
• Format: Journal Article
• Language: English
• Published: Chichester, UK John Wiley & Sons, Ltd 01.09.2024; Wiley Subscription Services, Inc
• Subjects: Animal models; Animals; Bile ducts; Bile Ducts - pathology; Cholangiocarcinoma; Cilia; Cilia - metabolism; Cilia - pathology; cystic liver; DEAD-box RNA Helicases - deficiency; DEAD-box RNA Helicases - genetics; DICER1 syndrome; Disease Models, Animal; Epithelium; Fibrosis; Hepatocellular carcinoma; Hyperplasia; intrahepatic cholangiocarcinoma; Liver; Liver - metabolism; Liver - pathology; Liver cancer; Liver Neoplasms - genetics; Liver Neoplasms - pathology; Mice; microRNA; Molecular modelling; mouse model; Mutation; primary cilia; Ribonuclease III - genetics; Ribonuclease III - metabolism; Tumorigenesis; Tumors; mouse model; primary cilia; cystic liver; microRNA; DICER1 syndrome; hepatocellular carcinoma; intrahepatic cholangiocarcinoma

DICER1 syndrome is a tumor predisposition syndrome caused by familial genetic mutations in DICER1. Pathogenic variants of DICER1 have been discovered in many rare cancers, including cystic liver tumors. However, the molecular mechanisms underlying liver lesions induced by these variants remain uncle...