Analysis Tools
normal phenotype
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• mice are viable with no detectable phenotypes
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Allele Symbol Allele Name Allele ID |
Arl13btm1c(EUCOMM)Wtsi targeted mutation 1c, Wellcome Trust Sanger Institute MGI:5907220 |
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| Summary |
2 genotypes
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• mice are viable with no detectable phenotypes
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• mice die at around P60
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• at P14, most DBA-positive collecting duct epithelial cells are devoid of cilia, indicating a cilia biogenesis defect
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• at P14, kidneys exhibit ~14% of BrdU-positive nuclei in DBA-positive cyst-lining cells versus ~2.8% in control kidneys, suggesting a significant increase in cell proliferation
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• mice show rapid kidney cyst formation, with minimal cysts at P7, significant cysts in both the medulla and cortex region at P14, and severely cystic kidneys at P17
• cyst formation is confined to the distal nephron
• at P14, cysts mainly form in the collecting duct; by P28, cysts are detected in the medullary thick ascending limb and the distal convoluted tubule
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• significant cysts in the cortex region at P14
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• significant cysts in the medulla region at P14
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• mice exhibit rapid progression of polycystic kidney disease (PKD) with severely cystic kidneys at P17
• treatment with valproic acid (VPA, a histone deacetylase inhibitor) can partially suppress PKD progression
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• starting at P14, kidney size is obviously enlarged; by P17, kidney size is significantly increased
• mice treated with valproic acid (VPA, a histone deacetylase inhibitor) from P10 to P25 show significantly reduced kidney size relative to vehicle-treated controls
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• at P14, kidney-to-body weight ratio is significantly increased
• mice treated with VPA from P10 to P25 show a significantly reduced kidney-to-body weight ratio relative to vehicle-treated controls
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• at P7, some DBA-positive regions marking the collecting ducts are already dilated
• in dilated regions, cilia are largely absent, whereas cilia are still present in non-dilated DBA-positive regions
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• at P28, but not at P21, kidneys exhibit increased trichrome staining indicating collagen deposition
• mice treated with VPA from P10 to P25 show a significantly reduced collagen deposition relative to vehicle-treated controls
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• at P28, kidney interstitial cells show a dramatic increase in smooth muscle actin expression relative to controls
• VPA treatment from P10 to P25 significantly reduces smooth muscle actin expression in the interstitium
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• mice develop rapidly progressive renal failure
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• BUN level is significantly increased at P14
• mice treated with VPA from P10 to P25 show a significantly reduced BUN level, indicating improved kidney function
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• at P14, most DBA-positive collecting duct epithelial cells are devoid of cilia, indicating a cilia biogenesis defect
|
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• at P14, kidneys exhibit ~14% of BrdU-positive nuclei in DBA-positive cyst-lining cells versus ~2.8% in control kidneys, suggesting a significant increase in cell proliferation
|
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• mice show rapid kidney cyst formation, with minimal cysts at P7, significant cysts in both the medulla and cortex region at P14, and severely cystic kidneys at P17
• cyst formation is confined to the distal nephron
• at P14, cysts mainly form in the collecting duct; by P28, cysts are detected in the medullary thick ascending limb and the distal convoluted tubule
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• significant cysts in the cortex region at P14
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• significant cysts in the medulla region at P14
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• mice exhibit rapid progression of polycystic kidney disease (PKD) with severely cystic kidneys at P17
• treatment with valproic acid (VPA, a histone deacetylase inhibitor) can partially suppress PKD progression
|
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• starting at P14, kidney size is obviously enlarged; by P17, kidney size is significantly increased
• mice treated with valproic acid (VPA, a histone deacetylase inhibitor) from P10 to P25 show significantly reduced kidney size relative to vehicle-treated controls
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• at P14, kidney-to-body weight ratio is significantly increased
• mice treated with VPA from P10 to P25 show a significantly reduced kidney-to-body weight ratio relative to vehicle-treated controls
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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 04/23/2024 MGI 6.23 |
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