In the last 25 years, a number of animal models, mainly rodents, have been generated with
the goal to mimic cholestatic liver injuries and, thus, to provide in vivo tools to investigate the …

Autosomal recessive polycystic kidney disease (ARPKD) is a hepatorenal fibrocystic
disorder that is characterized by enlarged kidneys with progressive loss of renal function …

Autosomal dominant polycystic liver disease results from mutations in PRKCSH or SEC63.
The respective gene products, glucosidase IIβ and SEC63p, function in protein translocation …

Autosomal recessive polycystic kidney disease (ARPKD) is an important cause of chronic
kidney disease in children. The care of ARPKD patients has traditionally been the realm of …

Emerging studies have suggested that the Hippo pathway is involved in the tumorigenesis of
hepatocellular carcinoma (HCC). However, the key regulator of the Hippo pathway in liver …

Autosomal dominant polycystic kidney disease (ADPKD) is the most common potentially
lethal monogenic disorder, with more than 12 million cases worldwide. The two causative …

Primary ciliary dyskinesia (PCD) is a rare genetically heterogeneous disorder caused by the
abnormal structure and/or function of motile cilia. The PCD diagnosis is challenging and …

Congenital hepatic fibrosis (CHF) is a disease of the biliary epithelium characterized by bile
duct changes resembling ductal plate malformations and by progressive peribiliary fibrosis …

The biliary tract consists of a network of intrahepatic and extrahepatic ducts that collect and
drain the bile produced by hepatocytes to the gut. The bile ducts are lined by …

Polycystic kidney disease (PKD) can arise from either developmental or postdevelopmental
processes. Recessive PKD, caused by mutations in PKHD1, is a developmental defect …