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Phenotypes Associated with This Genotype
Genotype
MGI:3815328
Allelic
Composition
Tns2nph/Tns2nph
Genetic
Background
involves: ICR
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tns2nph mutation (4 available); any Tns2 mutation (92 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• mice develop kidney disease around 40 to 150 days after birth and death occurs within two months of onset

reproductive system
• in severely affected mice the urogenitals look pale and watery
• mice exhibit reduced fertility although a line could be maintained as long as females unaffected by the kidney disease were used for mating

renal/urinary system
• in severely affected mice the urogenitals look pale and watery
• mice develop kidney disease around 40 to 150 days after birth and death occurs within two months of onset (J:24620)
• the kidney surface is smooth but becomes irregular as the disease progresses (J:24698)
• at early stages mice exhibit thickening of the capillary loops basement membrane and enlargement of the mesangial area compared to in wild-type mice
• thickening of the basement membrane continues as the disease progresses
• mice exhibit glomerular lesions with granular depositions that stain positive for IgA, IgG and IgM and in severe cases glomerular C3 staining depositions are observed
• in later stages mice exhibit dilation of the lumina tubules with lymphocyte infiltration around the small arteries unlike in wild-type mice

homeostasis/metabolism
• mice exhibit subcutaneous edemas in the trunk with varying decrees of chylaemia

hematopoietic system

immune system
• occasionally

adipose tissue
• little to no adipose tissue is present in the abdominal cavity in severely affected mice

digestive/alimentary system
• in severely affected mice the intestines look pale and watery

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
Pierson syndrome DOID:0060852 OMIM:609049
J:108691


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