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

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...

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Veröffentlicht in:	The Journal of pathology 2024-09, Vol.264 (1), p.17-29
Hauptverfasser:	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:	Artikel
Sprache:	eng
Schlagworte:	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
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