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Phenotypes Associated with This Genotype
Genotype
MGI:5430604
Allelic
Composition
Pkd1tm2Ggg/Pkd1tm2Ggg
Tg(Nes-cre)Wme/0
Genetic
Background
involves: 129S4/SvJae * C57BL/6 * CBA
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pkd1tm2Ggg mutation (1 available); any Pkd1 mutation (65 available)
Tg(Nes-cre)Wme mutation (0 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
renal/urinary system
• cyst-lining epithelial cells are highly apoptotic
• cyst-lining epithelial cells are highly proliferative
• -morphologically normal tubules and interstitial cells exhibit increased proliferation
• kidneys show extensive deposition of collagen
• kidneys are enlarged in 49 day old mutants
• mutants develop renal cysts by 49 days of age
• renal cysts arise predominately from collecting duct/distal tubules
• mutants treated with rapamycin exhibit an inhibition of renal cyst growth, a decrease in plasma blood urea nitrogen, fibrosis, and proliferation of cystic cells but show an increases cystic cell apoptosis (but not in normal tubule epithelial cells)

homeostasis/metabolism
• mutants exhibit highly elevated blood urea nitrogen

cellular
• cyst-lining epithelial cells are highly apoptotic
• cyst-lining epithelial cells are highly proliferative
• -morphologically normal tubules and interstitial cells exhibit increased proliferation

Mouse Models of Human Disease
OMIM ID Ref(s)
Polycystic Kidney Disease 1; PKD1 173900 J:185855


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
06/22/2016
MGI 6.04
The Jackson Laboratory