Phenotypes Associated with This Genotype

Genotype
MGI:6317329

• Allelic Composition: Pkd2^tm3Som/Pkd2^tm3Som; Tg(Pkhd1-cre)1Igr/0
• Genetic Background: involves: 129S4/SvJae * C57BL/6

• ♀: phenotype observed in females
• ♂: phenotype observed in males
• N: normal phenotype

mortality/aging

premature death ( J:244067 )

• median survival of approximately 70 days

renal/urinary system

increased kidney cell proliferation ( J:244067 )

• increase in cyst epithelial cell proliferation

• mice supplemented with the PPAR-alpha agonist fenofibrate or treated with an anti-miR-17 oligonucleotide show reduced cyst epithelial cell proliferation

polycystic kidney ( J:244067 )

• mice exhibit less aggressive polycystic kidney disease than Pkd1 mutants

• mice supplemented with the PPAR-alpha agonist fenofibrate show reduced cyst index

enlarged kidney ( J:244067 )

• kidney is enlarged due to cysts

• mice supplemented with the PPAR-alpha agonist fenofibrate or treated with an anti-miR-17 oligonucleotide show reduced kidney weight-to-body weight ratios

cellular

increased kidney cell proliferation ( J:244067 )

• increase in cyst epithelial cell proliferation

• mice supplemented with the PPAR-alpha agonist fenofibrate or treated with an anti-miR-17 oligonucleotide show reduced cyst epithelial cell proliferation

homeostasis/metabolism

increased circulating creatinine level ( J:244067 )

increased blood urea nitrogen level ( J:244067 )

• BUN levels are increased

• mice treated with an anti-miR-17 oligonucleotide show reduced BUN levels

growth/size/body

polycystic kidney ( J:244067 )

• mice exhibit less aggressive polycystic kidney disease than Pkd1 mutants

• mice supplemented with the PPAR-alpha agonist fenofibrate show reduced cyst index

enlarged kidney ( J:244067 )

• kidney is enlarged due to cysts

• mice supplemented with the PPAR-alpha agonist fenofibrate or treated with an anti-miR-17 oligonucleotide show reduced kidney weight-to-body weight ratios

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
polycystic kidney disease 2		DOID:0110859	OMIM:613095	J:244067