A missense mutation in the proprotein convertase gene furinb causes hepatic cystogenesis during liver development in zebrafish.

ABSTRACT: Hepatic cysts are fluid-filled lesions in the liver that are estimated to occur in 5% of the population. They may cause hepatomegaly and abdominal pain. Progression to secondary fibrosis, cirrhosis, or cholangiocarcinoma can lead to morbidity and mortality. Previous studies of patients and rodent models have associated hepatic cyst formation with increased proliferation and fluid secretion in cholangiocytes, which are partially due to impaired primary cilia. Congenital hepatic cysts are thought to originate from faulty bile duct development, but the underlying mechanisms are not fully understood. In a forward genetic screen, we identified a zebrafish mutant that developed hepatic cysts during larval stages. The cyst formation was not due to changes in biliary cell proliferation, bile secre

SUBMITTER: Ellis JL 

PROVIDER: S-EPMC9592797 | biostudies-literature | 2022 Nov

REPOSITORIES: biostudies-literature