Mouse Genome Informatics
hm
    Cys1cpk/Cys1cpk
C.B6(Cg)-Cys1cpk
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
renal/urinary system
• rapid kidney enlargement after birth associated with development of cysts in the collecting ducts
• EGF treatment reduced kidney size but did not improve kidney function
• after birth cysts shift to the collecting ducts and cysts are lined with a single layer of epithelial cells with polygonal principal-like cell appearance
• EGF treatment reduces the severity of kidney cysts
• newborns have cysts in the inner cortical proximal tubules

homeostasis/metabolism
• significant by 10 days of age

endocrine/exocrine glands
• dilation of the pancreatic ducts present in newborns
• intrahepatic biliary duct cysts associated with peridcuctal hyperplasia and fibrosis
• common bile duct dilation
• EGF treatment reduces bile duct dilation
• almost no islets of Langerhans seen
• periductal fibrosis

liver/biliary system
• intrahepatic biliary duct cysts associated with peridcuctal hyperplasia and fibrosis
• common bile duct dilation
• EGF treatment reduces bile duct dilation
• peridcuctal fibrosis associated with intrahepatic biliary duct cysts

growth/size
• seen at 5 and 15 days of age

digestive/alimentary system
• dilation of the pancreatic ducts present in newborns

Mouse Models of Human Disease
OMIM IDRef(s)
Polycystic Kidney Disease, Autosomal Recessive; ARPKD 263200 J:99616