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Phenotypes Associated with This Genotype
Genotype
MGI:3796072
Allelic
Composition
Glis2tm1Amj/Glis2tm1Amj
Genetic
Background
involves: 129P2/OlaHsd * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Glis2tm1Amj mutation (0 available); any Glis2 mutation (102 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• 40% of mice have died by 10 months of age compared to 5% of wild-type mice

renal/urinary system
• there is a five-fold higher increase in the number of renal tubules cells going through apoptosis
• the urine of some 10-month old mice contains many proteins including transferrin, albumin, and serpine
• by one year of age, 35% of mice have developed proteinuria
• focal lesions are first observed in the proximal tubules and glomeruli of the outer cortices of mutant mice
• by 3 to 4 months of age, the lesions progress into the inner cortex and medulla and are associated with inflammatory infiltrates and some interstitial fibrosis
• by 5 to 8 months of age, mononuclear inflammatory infiltrates are more extensive and are especially prominent around the vasculature
• by 8 to 12 months of age, the changes in the cortex are moderately to markedly severe and cover the majority of the cortex
• thickening of the basement membrane of Bowman's capsule at 12 months
• dilation of Bowman's space is observed at 5-8 months of age
• more pronounced dilation of Bowman's space by 12 months of age
• glomerular sclerosis by 12 months of age
• extensive fibrosis is associated with the glomerulonephritis that occurs in these mice
• kidneys of six month old mice weigh one third less (female) or one half (male) than wild-type mice
• the kidneys of adult mice are smaller than their wild-type controls and progressively atrophic with age
• atrophy of the capillary tuft is noted by 12 months of age
• at P25, affected proximal tubules are atrophic and lined by cuboidal basophilic epithelial cells
• by 12 months, atrophy and loss of the proximal tubules is noted in areas of fibrosis
• dilation of some cortical and medullary tubules is observed at 5-8 months of age
• tubules are variably dilated by 8-12 months of age
• at 5-8 months of age, several dilated renal tubules contain protein
• many tubules contain protein by 8-12 months of age
• mice exhibit progressive degeneration and increased cell death in the kidneys
• the kidneys of adult mice are paler than their wild-type controls
• mice develop progressive chronic kidney disease that resembles nephronophthisis and results in renal failure
• urine output increases with the impairment of kidneys

behavior/neurological
• increased water consumption at 12 months of age

homeostasis/metabolism
• mice as young as three months of age exhibit elevated levels of creatinine and these levels increase steadily with age
• mice as young as three months of age exhibit elevated levels of BUN and these levels increase steadily with age
• mice with high levels of BUN often died within days of analysis
• the urine of some 10-month old mice contains many proteins including transferrin, albumin, and serpine
• by one year of age, 35% of mice have developed proteinuria

immune system
• focal lesions are first observed in the proximal tubules and glomeruli of the outer cortices of mutant mice
• by 3 to 4 months of age, the lesions progress into the inner cortex and medulla and are associated with inflammatory infiltrates and some interstitial fibrosis
• by 5 to 8 months of age, mononuclear inflammatory infiltrates are more extensive and are especially prominent around the vasculature
• by 8 to 12 months of age, the changes in the cortex are moderately to markedly severe and cover the majority of the cortex

cellular
• there is a five-fold higher increase in the number of renal tubules cells going through apoptosis


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
12/10/2024
MGI 6.24
The Jackson Laboratory