Mouse Genome Informatics
hm
    Pkhd1tm2Cjwa/Pkhd1tm2Cjwa
either: B6.129(Cg)-Pkhd1tm2Cjwa or C.129(Cg)-Pkhd1tm2Cjwa
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
liver/biliary system
• visible by histology at 1 month of age
• significant increase in liver to body weight ratios in both sexes at 6, 9 and 12 months of age as compared to Pkhd1tm2.1Cjwa mice
• liver is completely replaced by cysts at 12 months of age
• mice develop progressive polycystic liver disease beginning at 3 months of age

renal/urinary system
• significant increase in kidney to body weight ratios in both sexes at 9 months of age as compared to Pkhd1tm2.1Cjwa mice
• significant increase in kidney to body weight ratios in females, but not males at 12 months of age as compared to Pkhd1tm2.1Cjwa mice
• females, but not males, develop proximal tubule dilation at 3 to 6 months of age (J:187735)
• only observed in females (J:187735)

endocrine/exocrine glands
N
• pancreas to body weight ratio is not significant for any timepoint when compared to Pkhd1tm2.1Cjwa mice (J:187735)

homeostasis/metabolism
• visible by histology at 1 month of age

Mouse Models of Human Disease
OMIM IDRef(s)
Polycystic Kidney Disease, Autosomal Recessive; ARPKD 263200 J:187735