Mouse Genome Informatics
hm
    Cys1cpk/Cys1cpk
D2J.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
• kidneys are grossly enlarged
• kidney wet weight is increased to 1.96g - 2.12g compared to 0.16g - 0.19g in wild-type mice
• multiple cysts of varying size that obscure the demarcation between the cortex and medulla
• cysts become larger with age and are initially found in the proximal tubules epithelial intercellular spaces are often focally ballooned

homeostasis/metabolism
• mean creatinine is 0.66mg% compared to 0.23mg% in wild-type mice
• mean BUN is 143mg% compared to 21mg% in wild-type mice

endocrine/exocrine glands
• poorly-formed acini are connected to enlarged, dilated ducts
• exocrine cells containing zygomen granules are seen in clusters unassociated with well-formed acini
• poorly-formed acini are connected to enlarged, dilated ducts
• bile ductules are somewhat enlarged
• few if any well-formed islets are found
• normal architecture is replaced by sheets of fibrous tissue

liver/biliary system
• bile ductules are somewhat enlarged
• some portal areas have increased fibrous tissue

behavior/neurological
• feed poorly during the first few hours of life and generally fail to thrive

digestive/alimentary system
• poorly-formed acini are connected to enlarged, dilated ducts
• exocrine cells containing zygomen granules are seen in clusters unassociated with well-formed acini
• poorly-formed acini are connected to enlarged, dilated ducts

Mouse Models of Human Disease
OMIM IDRef(s)
Polycystic Kidney Disease, Autosomal Recessive; ARPKD 263200 J:99620