Analysis Tools
renal/urinary system
|
• Background Sensitivity: dilation is detected in proximal tubules of congenic female mice by 3 months of age compared to 9 months of age in mice on a mixed background
|
|
Allele Symbol Allele Name Allele ID |
Pkhd1tm1Cjwa targeted mutation 1, Christopher J Ward MGI:3814174 |
||||||||||||||||
| Summary |
3 genotypes
|
|
|
| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
|
• Background Sensitivity: dilation is detected in proximal tubules of congenic female mice by 3 months of age compared to 9 months of age in mice on a mixed background
|
|
|
| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
|
• attenuation of the brush border is seen in females that develop cystic changes at 3 months of age
|
|
• Background Sensitivity: dilation is detected in proximal tubules of congenic female mice by 3 months of age compared to 9 months of age in mice on a mixed background
|
|
|
| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
 |
• females begin to develop cystic dilation in the outer stripe of the outer medulla and inner cortex at around 9 months of age
|
|
|
• in older female mice in dilated tubules the brush border is attenuated or in extreme cases lost
• in females at 9 months of age, surface blebs are seen on cilia and some cilia appear as branched clumps
• in female mice at 15 months of age cilia on the lining of cysts are usually stunted and highly branched
|
|
|
• dilation is confined to the proximal tubules in females at 9 - 15 months of age
• dilation in females increases from 9 to 12 months of age
• Background Sensitivity: dilation occurs later on a mixed background compared to congenic mice on either BALB/cJ or C57BL/6J backgrounds
• dilation is not seen in males at up to 18 months of age
|
|
• primary cilia are shorter and often misshapen with multiple blebs in biliary trees affected by cystic changes
|
|
• in early stages proliferation is seen in portal areas as primitive tortuous ducts and reactive stroma
|
|
• seen in all mice by 1 month of age
• worsens with age
|
|
• develop fibrocystic liver disease
• by 12 - 15 months of age, most of the liver is replaced with cysts
|
|
• increase in the liver to body weight ratio by 9 months of age
• livers are often massively enlarged occupying much of the abdominal cavity
|
|
• develop fibrocystic liver disease
• by 6 - 9 months of age, portal fibrosis is evident
|
|
• duct walls are thickened and lined with hyperplastic columnar epithelium
|
|
• ducts are dilated
|
|
• primary cilia are shorter and often misshapen with multiple blebs in biliary trees affected by cystic changes
|
|
• duct walls are thickened and lined with hyperplastic columnar epithelium
|
|
• ducts are dilated
|
|
• in early stages proliferation is seen in portal areas as primitive tortuous ducts and reactive stroma
|
|
• seen in all mice by 1 month of age
• worsens with age
|
|
• in some mice the pancreas is enlarged and markedly cystic
• dilated cysts are lined with hyperplastic columnar epithelium surrounded by fibrotic stroma
• microscopic cysts were seen in 44 of 92 mice
|
|
• primary cilia are shorter and often misshapen with multiple blebs in biliary trees affected by cystic changes
|
|
• in some mice the pancreas is enlarged and markedly cystic
• dilated cysts are lined with hyperplastic columnar epithelium surrounded by fibrotic stroma
• microscopic cysts were seen in 44 of 92 mice
|
|
|
• females begin to develop cystic dilation in the outer stripe of the outer medulla and inner cortex at around 9 months of age
|
|
• develop fibrocystic liver disease
• by 12 - 15 months of age, most of the liver is replaced with cysts
|
|
• increase in the liver to body weight ratio by 9 months of age
• livers are often massively enlarged occupying much of the abdominal cavity
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| autosomal recessive polycystic kidney disease | DOID:0110861 |
OMIM:263200 |
J:140302 | |
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO) |
||
|
Citing These Resources Funding Information Warranty Disclaimer, Privacy Notice, Licensing, & Copyright Send questions and comments to User Support. |
last database update 04/16/2024 MGI 6.23 |
|
|
|
||