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Phenotypes Associated with This Genotype
Genotype
MGI:5792829
Allelic
Composition
Dicer1tm1Smr/Dicer1tm1Smr
Pax8tm1.1(cre)Mbu/Pax8+
Genetic
Background
involves: 129P2/OlaHsd * 129S7/SvEvBrd
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Dicer1tm1Smr mutation (1 available); any Dicer1 mutation (96 available)
Pax8tm1.1(cre)Mbu mutation (1 available); any Pax8 mutation (32 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
renal/urinary system
• apoptosis is frequently seen in shedding cells in both the Bowman space and in the lumen of the tubules, occurring more frequently at P50
• the tubular epithelium and parietal cells of the renal corpuscles show increased cellular proliferation in the phase of cyst growth
• mice develop progressively massive proteinuria, consisting mainly of albuminuria that is already higher at P30
• seen by P30
• at P30, urinary osmolality is lower, with no changes in urinary ouput or in collecting duct distribution
• kidneys at P50 have a rough surface
• at P50, primary cilia are rare in the epithelium of dilating tubules and absent in enlarged cysts with flattened epithelium, indicating a progressive loss of primary cilia with cyst enlargement
• at P50, mice exhibit cortical cysts
• cysts originate from the renal corpuscles of the cortex and are absent in the outer and inner medulla
• the density of collecting ducts is lower at P50
• the tubular epithelium and parietal cells of the renal corpuscles show increased cellular proliferation in the phase of cyst growth, before flattening of the cystic epithelium
• dilatation of the parietal cells of the Bowman capsule
• glomeruli progressively shrink
• mice develop glomerulocystic disease
• epithelium lining the cyst flattens and interstitial fibrosis develops
• mice present lower kidney weight at P50, with the ratio of kidney weight/body weight being lower at P50 but not P30
• tubular dilatations are mainly cortical and develop progressively at P50
• kidneys at P50 have a paler color
• progressive impairment of urinary concentrating ability
• increase in urine output at P50 but not P30

cellular
• at P50, primary cilia are rare in the epithelium of dilating tubules and absent in enlarged cysts with flattened epithelium, indicating a progressive loss of primary cilia with cyst enlargement
• apoptosis is frequently seen in shedding cells in both the Bowman space and in the lumen of the tubules, occurring more frequently at P50
• the tubular epithelium and parietal cells of the renal corpuscles show increased cellular proliferation in the phase of cyst growth

growth/size/body
• mice present lower body weight at P30
• at P50, mice exhibit cortical cysts
• cysts originate from the renal corpuscles of the cortex and are absent in the outer and inner medulla

homeostasis/metabolism
• mice develop progressively massive proteinuria, consisting mainly of albuminuria that is already higher at P30
• seen by P30
• at P30, urinary osmolality is lower, with no changes in urinary ouput or in collecting duct distribution

behavior/neurological
• increase in water intake at P30 and P50


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
01/06/2026
MGI 6.24
The Jackson Laboratory