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Phenotypes Associated with This Genotype
Genotype
MGI:5519084
Allelic
Composition
Tg(Pkd1*)39Mtru/0
Genetic
Background
involves: C57BL/6J * CBA/J
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No mouse lines available in IMSR.
See publication links below for author information.
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• mean lifespan is around 17.4 months of age compared to 23-29 months in controls

renal/urinary system
• non-selective proteinuria that is detectable at 12 months of age and increases further with age
• glomerular tufts are hypocellular and become sclerotic
• renal parenchyma shows presence of mild to extensive interstitial fibrosis with localized lymphoid infiltrates that are often perivascular
• renal parenchyma exhibits loss of tubules
• renal dilatation is first seen around 2 months of age
• very mild renal tubular dilatation is seen around 2 months of age that progresses into polycystic kidney (multiple macro-and micro-cysts) disease with age
• glomeruli exhibit cysts associated with hyperplasia of the parietal epithelial cells
• kidneys become pale over time with an embossed surface
• late-onset of renal failure
• 3-4 fold increase in urine volume, a concentrating defect that progresses with age

hematopoietic system

homeostasis/metabolism
• decrease in urine ion excretion
• non-selective proteinuria that is detectable at 12 months of age and increases further with age

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
polycystic kidney disease 1 DOID:0110858 OMIM:173900
J:198147


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB), Gene Ontology (GO)
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last database update
08/06/2019
MGI 6.14
The Jackson Laboratory