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Phenotypes Associated with This Genotype
Genotype
MGI:3583121
Allelic
Composition
Nphp3pcy/Nphp3pcy
Genetic
Background
KK-Nphp3pcy
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Nphp3pcy mutation (0 available); any Nphp3 mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• mean life span is about 186 days with a few homozygotes living to 9 months of age

renal/urinary system
• around cysts in the distal convoluted tubules focal fibrotic areas with chronic inflammatory infiltrates are seen; however neither papillary necrosis nor amyloidosis are seen
• visible at 3 months of age and in 25 weeks old homozygotes the kidneys occupy almost the entire abdominal cavity
• average kidneys weight at 25 weeks of age is 3.69+/-0.59 g and 3.35+/-0.31 g in males and females, respectively, compared to 0.35+/-0.01 g in control mice
• in newborns small cyst-like features are seen including distension of Bowman's capsules, proximal convoluted tubules, distal convoluted tubules, and collecting tubules
• by 25 weeks the cysts are seen on the surface of the kidney and in sections are seen in both the cortex and medulla distorting the cortico-medullary junction; however, no cysts are seen in the liver, pancreas, or lungs

homeostasis/metabolism
• by 15 weeks of age

hematopoietic system
• average kidneys weight at 25 weeks of age is 3.69+/-0.59 g and 3.35+/-0.31 g in males and females, respectively, compared to 0.35+/-0.01 g in control mice

behavior/neurological
• seen around 4 months of age
• seen around 4 months of age

growth/size/body
• seen at 3 months of age

immune system
• around cysts in the distal convoluted tubules focal fibrotic areas with chronic inflammatory infiltrates are seen; however neither papillary necrosis nor amyloidosis are seen

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
autosomal dominant polycystic kidney disease DOID:898 OMIM:PS173900
J:8302


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
02/12/2019
MGI 6.13
The Jackson Laboratory