Evidence for a “Pathogenic Triumvirate” in Congenital Hepatic Fibrosis in Autosomal Recessive Polycystic Kidney Disease

Table 1

Current rodent models of ARPKD.

Model	Species	Liver phenotype	Kidney phenotype	Other phenotypes	Reference
PCK	Rat	Cysts and fibrosis	Cysts	Pancreatic cysts	[29]
BALB/c-cpk/cpk	Mouse	Cysts and fibrosis	Cysts	Pancreatic cysts and fibrosis	[33]
C57BL/6J-cpk/cpk	Mouse	No liver disease	Cysts	None	[34]
	Mouse	Cysts and fibrosis	Cysts in female	Pancreatic cysts	[35]
	Mouse	Cysts and fibrosis	Cysts in female	Unknown	[36]
Pkhd1exon40	Mouse	Cysts and fibrosis	No kidney disease	Portal hypertension	[37]
	Mouse	Cysts and fibrosis	Cysts	Pancreatic and gall bladder cysts	[5]
	Mouse	Cysts and fibrosis	No kidney disease	Pancreatic cysts, splenomegaly	[38]