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Phenotypes Associated with This Genotype
Genotype
MGI:3583530
Allelic
Composition
Cys1cpk/Cys1cpk
Genetic
Background
involves: C57BL/6J * CD-1
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
mortality/aging
• uremic mortality is seen in less than 10% of homozygotes at 14-20 days of age (J:33739)
• uremic mortality is seen in less than 10% of homozygotes at 14-20 days of age (J:33739)

renal/urinary system
• seen at 4 days of age (J:33739)
• seen at 4 days of age (J:33739)
• at 4 days of age proximal tubule cysts are seen with focal collecting duct dilations in the cortex and outer medulla and by 8-20 days of age collecting duct cysts expand to replace almost all the normal kidney tissue (J:33739)
• cells lining the proximal tubule cysts and later the collecting duct cysts appear immature (J:33739)
• at 4 days of age proximal tubule cysts are seen with focal collecting duct dilations in the cortex and outer medulla and by 8-20 days of age collecting duct cysts expand to replace almost all the normal kidney tissue (J:33739)
• cells lining the proximal tubule cysts and later the collecting duct cysts appear immature (J:33739)

homeostasis/metabolism
• azotemia seen at 12 days of age (J:33739)
• azotemia seen at 12 days of age (J:33739)

endocrine/exocrine glands
• all common bile ducts have cysts (J:33739)
• all common bile ducts have cysts (J:33739)
• all pancreatic ducts have cysts (J:33739)
• all pancreatic ducts have cysts (J:33739)

liver/biliary system
• all common bile ducts have cysts (J:33739)
• all common bile ducts have cysts (J:33739)
• intrahepatic bile duct cysts consisting of focal dilations in the triad region through to the terminal intrahepatic branches without signs of significant periductal proliferation (J:33739)
• intrahepatic bile duct cysts consisting of focal dilations in the triad region through to the terminal intrahepatic branches without signs of significant periductal proliferation (J:33739)

immune system

hematopoietic system

Mouse Models of Human Disease
OMIM ID Ref(s)
Polycystic Kidney Disease, Autosomal Recessive; ARPKD 263200 J:33739


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
02/02/2016
MGI 6.02
The Jackson Laboratory