Mouse Genome Informatics
cn
    Pkd1tm3Jzh/Pkd1tm3Jzh
Tg(Ggt1-cre)M3Egn/0

involves: 129P2/OlaHsd * C57BL/6 * SJL
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
mortality/aging
• most mice die by one month of age from renal failure

renal/urinary system
• increased apoptosis is observed in both cysts and normal looking epithelial tubules of 2 day old mice
• cystic kidneys are first detectable at 10 days of age
• the disease is progressive with cysts dominating the kidney architecture by 26 days of age
• however, glomerular cysts are not observed
• cysts of proximal and distal origin develop at different rates with distal cysts progressively increasing in size until death
• proximal cysts stop growing at 15 days of age and start to regress or completely disappear before death

cellular
• increased apoptosis is observed in both cysts and normal looking epithelial tubules of 2 day old mice

Mouse Models of Human Disease
OMIM IDRef(s)
Polycystic Kidney Disease 1; PKD1 173900 J:140012