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Phenotypes Associated with This Genotype
Genotype
MGI:3583531
Allelic
Composition
Cys1cpk/Cys1cpk
Genetic
Background
C.B6(Cg)-Cys1cpk
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cys1cpk mutation (1 available); any Cys1 mutation (2 available)
phenotype observed in females
phenotype observed in males
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/body
• 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 ID Ref(s)
Polycystic Kidney Disease, Autosomal Recessive; ARPKD 263200 J:99616


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
04/26/2016
MGI 6.03
The Jackson Laboratory