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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Pkd2tm1.1Gwu
targeted mutation 1.1, Guanqing Wu
MGI:6201745
Summary 1 genotype
Jump to Allelic Composition Genetic Background Genotype ID
cn1
Pkd2tm1.1Gwu/Pkd2tm1.1Gwu
Tg(Vil1-cre)997Gum/0
involves: C57BL/6 * C57BL/6J * SJL MGI:6251480


Genotype
MGI:6251480
cn1
Allelic
Composition
Pkd2tm1.1Gwu/Pkd2tm1.1Gwu
Tg(Vil1-cre)997Gum/0
Genetic
Background
involves: C57BL/6 * C57BL/6J * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pkd2tm1.1Gwu mutation (0 available); any Pkd2 mutation (85 available)
Tg(Vil1-cre)997Gum mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• most mice die between 4 and 6 months of age
• more than 50% of females survive to 5 months of age, approximately 1 month longer than males, indicating better survival for females
• treatment with rapamycin from P10 to P60 improves survival, with female rapamycin-treated mice having a median survival of about 7 months and male rapamycin-treated mice 6 months
• enhanced rapamycin treatment (extended duration) improves lifespan even more to a median survival of about 8 months

renal/urinary system
• mice show increased proliferation in renal epithelia
• rapamycin treatment suppresses the increased proliferation in renal epithelia
• by 4 months of age, normal parenchyma is almost completely lost in the kidney
• mice develop severe cysts in the kidneys
• renal cysts derive from all nephronic segments of the kidney
• renal cytogenesis is more severe in males than females
• treatment with rapamycin from P10 to P60 reduces growth of renal cysts, with treatment response better in females than males
• enhanced rapamycin treatment (extended duration) reduces renal cyst growth even more
• mice exhibit a larger kidney/body weight ratio by P15

endocrine/exocrine glands
• cysts in the pancreas are seen starting at 4 months of age

liver/biliary system
• cysts in liver are seen starting at 2 months of age with the cystic liver worsening rapidly with age
• however, the liver/body weight ratio and alanine aminotransferase levels are not different from wild-type

homeostasis/metabolism
• mice show a higher creatinine level at 1 month of age
• creatinine levels are more elevated in males than in females at 4 months of age
• treatment with rapamycin improves creatinine levels but not completely to normal levels
• enhanced rapamycin treatment improves creatinine levels further
• mice show a higher blood urea nitrogen level at 2 months of age
• blood urea nitrogen levels are more elevated in males than in females at 4 months of age
• treatment with rapamycin or enhanced rapamycin (extended duration) improves BUN levels to normal

cellular
• mice show increased proliferation in renal epithelia
• rapamycin treatment suppresses the increased proliferation in renal epithelia

growth/size/body
• cysts in the pancreas are seen starting at 4 months of age
• mice develop severe cysts in the kidneys
• renal cysts derive from all nephronic segments of the kidney
• renal cytogenesis is more severe in males than females
• treatment with rapamycin from P10 to P60 reduces growth of renal cysts, with treatment response better in females than males
• enhanced rapamycin treatment (extended duration) reduces renal cyst growth even more
• cysts in liver are seen starting at 2 months of age with the cystic liver worsening rapidly with age
• however, the liver/body weight ratio and alanine aminotransferase levels are not different from wild-type
• mice exhibit a larger kidney/body weight ratio by P15

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
autosomal dominant polycystic kidney disease DOID:898 J:265516





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last database update
12/10/2024
MGI 6.24
The Jackson Laboratory