Phenotypes associated with this allele
|
Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pkd1tm1.1Pcha mutation
(0 available);
any
Pkd1 mutation
(153 available)
Pkd1tm2Som mutation
(0 available);
any
Pkd1 mutation
(153 available)
Tg(Cdh16-cre)91Igr mutation
(1 available)
|
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mortality/aging
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• median survival is 206 days
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renal/urinary system
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• mice develop polycystic kidney disease
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• mice show impaired renal function
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• increase in cyst epithelial cell proliferation
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cellular
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• the number of peroxisomes is decreased in kidney cysts
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• increase in cyst epithelial cell proliferation
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• fatty acid oxidation is decreased in kidneys
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• reactive oxidative species level is increased in the kidneys
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homeostasis/metabolism
growth/size/body
|
• mice develop polycystic kidney disease
|
|
Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mirc1tm1.1Tyj mutation
(1 available);
any
Mirc1 mutation
(6 available)
Pkd1tm1.1Pcha mutation
(0 available);
any
Pkd1 mutation
(153 available)
Pkd1tm2Som mutation
(0 available);
any
Pkd1 mutation
(153 available)
Tg(Cdh16-cre)91Igr mutation
(1 available)
|
|
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renal/urinary system
N |
• renal function and renal interstitial fibrosis are improved compared to conditional Pkd1 compound heterozygotes and mice live past 1 year of age
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• mice show a decrease in cyst epithelial cell proliferation compared to conditional Pkd1 compound heterozygotes
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• cyst size is reduced compared to conditional Pkd1 compound heterozygotes
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• kidney weight is reduced compared to conditional Pkd1 compound heterozygotes
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cellular
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• the number of peroxisomes in the kidneys is reduced compared to conditional Pkd1 compound heterozygotes
|
|
• mice show a decrease in cyst epithelial cell proliferation compared to conditional Pkd1 compound heterozygotes
|
|
• fatty acid oxidation is increased in kidneys compared to conditional Pkd1 compound heterozygotes
|
|
• reactive oxidative species level is decreased in the kidneys compared to conditional Pkd1 compound heterozygotes
|
homeostasis/metabolism
growth/size/body
|
• cyst size is reduced compared to conditional Pkd1 compound heterozygotes
|
|
Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mirc1tm1.1Tyj mutation
(1 available);
any
Mirc1 mutation
(6 available)
Pkd1tm2Som mutation
(0 available);
any
Pkd1 mutation
(153 available)
Tg(Cdh16-cre)91Igr mutation
(1 available)
|
|
|
mortality/aging
|
• mice exhibit a median survival of 21 days which is a 50% improvement compared to single Pkd1 conditional mutants
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renal/urinary system
|
• the number of proliferating cyst epithelial cells is reduced by 78.7% compared to single Pkd1 conditional mutants
|
|
• mice exhibit a 26.8% reduction in kidney-weight-to-body-weight ratio compared to single Pkd1 conditional mutants
|
cellular
|
• the number of proliferating cyst epithelial cells is reduced by 78.7% compared to single Pkd1 conditional mutants
|
homeostasis/metabolism
|
Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pkd1tm2Som mutation
(0 available);
any
Pkd1 mutation
(153 available)
Tg(Cdh16-cre)91Igr mutation
(1 available)
|
|
|
mortality/aging
|
• median survival is 14 days, with mice starting to die around 10 days and most die by 20 days of age
|
renal/urinary system
|
• increase in cyst epithelial cell proliferation
|
|
• mice develop an early-onset and rapidly fatal form of polycystic kidney disease
|
cellular
|
• increase in cyst epithelial cell proliferation
|
homeostasis/metabolism
growth/size/body
|
• mice develop an early-onset and rapidly fatal form of polycystic kidney disease
|
|
Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pkd1tm1Som mutation
(0 available);
any
Pkd1 mutation
(153 available)
Pkd1tm2Som mutation
(0 available);
any
Pkd1 mutation
(153 available)
Tg(Cdh16-cre/ERT2)24Igr mutation
(0 available)
|
|
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cellular
|
• loss of orientation of cell division to the lumen of the medullary collecting ducts of the kidney starts to develop after tubules begin to dilate
• no significant change in orientation of cell division to the lumen of the collecting ducts of the kidney at 3 days of age
|
|
Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pkd1tm2.1Som mutation
(0 available);
any
Pkd1 mutation
(153 available)
Pkd1tm2Som mutation
(0 available);
any
Pkd1 mutation
(153 available)
Tg(Cdh16-cre)91Igr mutation
(1 available)
|
|
|
mortality/aging
|
• mice die between P14 and P17 of renal failure
|
renal/urinary system
|
• at P7, proliferation of the cyst lining cells in the kidney is increased compared to in wild-type mice
|
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• mice exhibit a rapid progression of polycystic disease
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• an increased kidney weight to body weight ratio is observed at day 4 and increases rapidly thereafter
|
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• mice die between P14 and P17 of renal failure
|
homeostasis/metabolism
growth/size/body
|
• mice exhibit a rapid progression of polycystic disease
|
|
• an increased kidney weight to body weight ratio is observed at day 4 and increases rapidly thereafter
|
|
Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pkd1tm2Som mutation
(0 available);
any
Pkd1 mutation
(153 available)
Tg(Cdh16-cre)91Igr mutation
(1 available)
|
|
|
mortality/aging
|
• mice die between P14 and P17 of renal failure
|
renal/urinary system
|
• at P7, proliferation of the cyst lining cells in the kidney is increased compared to in wild-type mice
|
|
• mice exhibit a rapid progression of polycystic disease
|
|
• an increased kidney weight to body weight ratio is observed at day 4 and increases rapidly thereafter
|
|
• mice die between P14 and P17 of renal failure
|
homeostasis/metabolism
growth/size/body
|
• mice exhibit a rapid progression of polycystic disease
|
|
• an increased kidney weight to body weight ratio is observed at day 4 and increases rapidly thereafter
|
|
Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pkd1tm2Som mutation
(0 available);
any
Pkd1 mutation
(153 available)
Tg(Pkhd1-cre)1Igr mutation
(0 available)
|
|
|
renal/urinary system
|
• at P24, proliferation of the cyst lining cells in the kidney is increased compared to in wild-type mice
|
growth/size/body