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Phenotypes Associated with This Genotype
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cys1cpk mutation (1 available); any Cys1 mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
• die by 20-23 days of age (J:6718)
• die around 19 days of age (J:31533)

renal/urinary system
• urine is colorless in grossly affected mice
• large cysts distort the corticomedullary junction
• at 23 days of age, kidneys occupy almost the entire abdominal cavity (J:6718)
• at 17-24 days of age, average kidneys size was 16 mm x 11 mm compared to 6 mm x 3 mm in wild-type mice (J:6718)
• increase in kidney size is the result of both increased cystic fluid and increased cell mass (J:9290)
• kidney weight is increased in newborns and a progressive increase in kidney wet and dry weight as well as size is seen with age
• large cysts occupy the entire kidney, distort the corticomedullary junction, involve collecting ducts and other portions of the kidney, and are lined with flattened epithelium the is frequently squamous (J:6718)
• initially presents as dilation of the proximal tubule in fetal and newborn mice, then shifts to dilation of the cortical and medullary collecting ducts after the first week of life (J:9290)
• epithelial hyperplasia but not polypoid hyperplasia is seen in the cysts (J:9290)
• bilateral polycystic kidneys become apparent by 8 - 9 days of age

• mean BUN was 120mg% compared to 0.2mg% in wild-type mice (J:6718)
• progressive azotemia seen starting at 1 week of age (J:9290)
• urine is colorless in grossly affected mice

• seen at 10-13 days of age (J:6718)
• apparent by 8 - 9 days of age (J:31533)

hematopoietic system
• seen at 2 - 3 weeks of age

• become progressively lethargic

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
autosomal recessive polycystic kidney disease DOID:0110861 OMIM:263200
J:6718 , J:9290

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Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
MGI 6.08
The Jackson Laboratory