ArticleThe American journal of pathology2001
Polycystic kidney rat is a novel animal model of Caroli's disease associated with congenital hepatic fibrosis.
Article in The American journal of pathology, 2001. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 29 papers.
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Who cites it
29 citing papers in PubMed, 87 citations in OpenAlex.
- Review
- Review of the Use of Animal Models of Human Polycystic Kidney Disease for the Evaluation of Experimental Therapeutic Modalities.Journal of clinical medicine · 2023Review
- Review
- Mitigation of portal fibrosis and cholestatic liver disease in ANKS6-deficient livers by macrophage depletion.FASEB journal : official publication of the Federation of American Societies for Experimental Biology · 2022Article
- Remodeling of Intrahepatic Ducts in a Model of Caroli Syndrome: Is Scar Carcinoma a Consequence of Laplace's Law?Medical sciences (Basel, Switzerland) · 2019Article
- Animal models of biliary injury and altered bile acid metabolism.Biochimica et biophysica acta. Molecular basis of disease · 2018Review
- Article
- Initial evaluation of hepatic T1 relaxation time as an imaging marker of liver disease associated with autosomal recessive polycystic kidney disease (ARPKD).NMR in biomedicine · 2016Article
- Article
- Activation of the PI3K/mTOR pathway is involved in cystic proliferation of cholangiocytes of the PCK rat.PloS one · 2014Article
- Article
- What are the precursor and early lesions of peripheral intrahepatic cholangiocarcinoma?International journal of hepatology · 2014Review
- α1- and α5-containing laminins regulate the development of bile ducts via β1 integrin signals.The Journal of biological chemistry · 2012Article
- Caroli's Disease: Current Knowledge of Its Biliary Pathogenesis Obtained from an Orthologous Rat Model.International journal of hepatology · 2012Article
- Establishment of a standardized liver fibrosis model with different pathological stages in rats.Gastroenterology research and practice · 2012Article
- Congenital hepatic fibrosis in autosomal recessive polycystic kidney disease.Clinical and translational science · 2011Review
- Biliary infection may exacerbate biliary cystogenesis through the induction of VEGF in cholangiocytes of the polycystic kidney (PCK) rat.The American journal of pathology · 2011Article
- Disease stage characterization of hepatorenal fibrocystic pathology in the PCK rat model of ARPKD.Anatomical record (Hoboken, N.J. : 2007) · 2010Article
- Renin-angiotensin system activation in congenital hepatic fibrosis in the PCK rat model of autosomal recessive polycystic kidney disease.Journal of pediatric gastroenterology and nutrition · 2010Article
- Polycystic liver diseases.Digestive and liver disease : official journal of the Italian Society of Gastroenterology and the Italian Association for the Study of the Liver · 2010Review
Corrections and comments
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Authors and funding
6 authors at 3 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Caroli's disease (congenital intrahepatic biliary dilatation) associated with congenital hepatic fibrosis is an autosomal recessive polycystic kidney disease. Recently, the polycystic kidney (PCK) rat, a spontaneous mutant derived from a colony of CRJ:CD rats with polycystic lesions in the liver and an autosomal recessive mode of inheritance, was reported. In the present study, the pathology of the hepatobiliary system and the biliary cell-kinetics were evaluated in fetuses (day 18 to 21 of gestation) and neonates and adults (1 day to 4 months after delivery) of PCK rats. CRJ:CD rats were used as a control. Multiple segmental and saccular dilatations of intrahepatic bile ducts were first observed in fetuses at 19 days of gestation. The dilatation spread throughout the liver and the degree of dilatation increased with aging. Gross and histological features characterizing ductal plate malformation were common in the intrahepatic bile ducts. Overgrowth of portal connective tissue was evident and progressive after delivery. These features were very similar to those of Caroli's disease with congenital hepatic fibrosis. Proliferative activity in the biliary epithelial cells was greater in PCK rats than controls during the development. In contrast, the biliary epithelial apoptosis was less extensive in PCK rats than the controls until 1 week after delivery, but greater after 3 weeks, suggesting that the remodeling defect in immature bile ducts associated with the imbalance of cell kinetics plays a role in the occurrence of intrahepatic biliary anomalies in PCK rats. The PCK rat could be a useful and promising animal model of Caroli's disease with congenital hepatic fibrosis.
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