Phenotypes Associated with This Genotype

Genotype
MGI:3795669

• Allelic Composition: Pkd1^tm2Som/Pkd1^tm2.1Som; Tg(Cdh16-cre)91Igr/0
• Genetic Background: involves: C57BL/6 * ICR * SJL

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

mortality/aging

postnatal lethality, complete penetrance ( J:135301 )

• mice die between P14 and P17 of renal failure

renal/urinary system

abnormal kidney morphology ( J:135301 )

• at P7, proliferation of the cyst lining cells in the kidney is increased compared to in wild-type mice

polycystic kidney ( J:135301 )

• mice exhibit a rapid progression of polycystic disease

increased kidney weight ( J:135301 )

• an increased kidney weight to body weight ratio is observed at day 4 and increases rapidly thereafter

kidney failure ( J:135301 )

• mice die between P14 and P17 of renal failure

homeostasis/metabolism

increased blood urea nitrogen level ( J:135301 )

• the rapid increase in blood urea nitrogen level is associated with the cystic enlargement of the kidneys

decreased circulating glucose level ( J:135301 )

• at two weeks of age

increased circulating cholesterol level ( J:135301 )

• at two weeks of age

increased circulating phosphate level ( J:135301 )

growth/size/body

polycystic kidney ( J:135301 )

• mice exhibit a rapid progression of polycystic disease

increased kidney weight ( J:135301 )

• an increased kidney weight to body weight ratio is observed at day 4 and increases rapidly thereafter

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
polycystic kidney disease 1		DOID:0110858	OMIM:173900	J:135301