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Phenotypes Associated with This Genotype
Genotype
MGI:4437766
Allelic
Composition
Tg(Pkd1)26Mtru/0
Genetic
Background
involves: C57BL/6J * CBA/J
Find Mice Using the International Mouse Strain Resource (IMSR)
No mouse lines available in IMSR.
See publication links below for author information.
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• mice die around 6 months of age (J:157952)
• mice die around 6 months of age (J:157952)

renal/urinary system
• proliferation of kidney cells is increased compared to in wild-type mice (J:157952)
• proliferation of kidney cells is increased compared to in wild-type mice (J:157952)
• at 5 to 7 months, urine protein levels are decreased compared to in wild-type mice (J:157952)
• at 5 to 7 months, urine protein levels are decreased compared to in wild-type mice (J:157952)
• at 4 months, mice exhibit non-selective proteinuria unlike wild-type mice (J:157952)
• at 4 months, mice exhibit non-selective proteinuria unlike wild-type mice (J:157952)
• partial and total (J:157952)
• partial and total (J:157952)
• cystic and non-cystic tubules exhibit epithelial hyperplasia and hypertrophy with occasional polyps of varying severity unlike wild-type mice (J:157952)
• cystic and non-cystic tubules exhibit epithelial hyperplasia and hypertrophy with occasional polyps of varying severity unlike wild-type mice (J:157952)
• at 1 month of age (J:157952)
• at 1 month of age (J:157952)
• at 2 to 8 months (J:157952)
• at 2 to 8 months (J:157952)
• at 1 month, mice exhibit mild tubular dilation unlike wild-type mice (J:157952)
• at 1 month, mice exhibit mild tubular dilation unlike wild-type mice (J:157952)
• proteinaceous casts in tubular cysts (J:157952)
• proteinaceous casts in tubular cysts (J:157952)
• at 1 month of age, mice exhibit scattered tubular microcysts unlike wild-type mice (J:157952)
• at 9 months, mice develop cysts unlike wild-type mice (J:157952)
• by 12 to 16 months, mice exhibit severe cysts unlike wild-type mice (J:157952)
• mice develop cysts in the medulla, cortex and glomeruli unlike wild-type mice (J:157952)
• mice develop hemorrhagic cysts unlike wild-type mice (J:157952)
• cysts exhibit proteinaceous casts in tubular cysts and interstitial fibrosis unlike wild-type mice (J:157952)
• cysts originate from the proximal and collecting ducts and glomeruli unlike in wild-type mice (J:157952)
• cysts exhibit calcium deposits (J:157952)
• at 1 month of age, mice exhibit scattered tubular microcysts unlike wild-type mice (J:157952)
• at 9 months, mice develop cysts unlike wild-type mice (J:157952)
• by 12 to 16 months, mice exhibit severe cysts unlike wild-type mice (J:157952)
• mice develop cysts in the medulla, cortex and glomeruli unlike wild-type mice (J:157952)
• mice develop hemorrhagic cysts unlike wild-type mice (J:157952)
• cysts exhibit proteinaceous casts in tubular cysts and interstitial fibrosis unlike wild-type mice (J:157952)
• cysts originate from the proximal and collecting ducts and glomeruli unlike in wild-type mice (J:157952)
• cysts exhibit calcium deposits (J:157952)
• bilateral (J:157952)
• bilateral (J:157952)
• calcium deposits were limited to the renal papilla (J:157952)
• calcium deposits were limited to the renal papilla (J:157952)
• at 5 to 7 months (J:157952)
• at 5 to 7 months (J:157952)

cardiovascular system
• altered ventricular vasculature patterns indicate myocardium injury unlike in wild-type mice (J:157952)
• altered ventricular vasculature patterns indicate myocardium injury unlike in wild-type mice (J:157952)
• thickness is increased (J:157952)
• thickness is increased (J:157952)
• mice exhibit evidence of eccentric dilated cardiac hypertrophy (J:157952)
• mice exhibit evidence of eccentric dilated cardiac hypertrophy (J:157952)
• left ventricular wall systolic and diastolic thickness are increased compared to in wild-type mice indicating hypertrophy (J:157952)
• left ventricular wall systolic and diastolic thickness are increased compared to in wild-type mice indicating hypertrophy (J:157952)
• aortic valve leaflets are opaque rather than translucent as in wild-type mice (J:157952)
• aortic valve leaflets are opaque rather than translucent as in wild-type mice (J:157952)
• an increase in mean and peak velocity downstream of the aortic valve suggests stenosis (J:157952)
• an increase in mean and peak velocity downstream of the aortic valve suggests stenosis (J:157952)
• 6 of 15 aortic valves indicate calcification unlike in wild-type mice (J:157952)
• 6 of 15 aortic valves indicate calcification unlike in wild-type mice (J:157952)
• left ventricular wall systolic and diastolic thickness are increased compared to in wild-type mice indicating hypertrophy (J:157952)
• left ventricular wall systolic and diastolic thickness are increased compared to in wild-type mice indicating hypertrophy (J:157952)
• 35% to 40% of mice exhibit a 2- to 4-fold increase in cardiac fibrosis compared with wild-type mice (J:157952)
• 35% to 40% of mice exhibit a 2- to 4-fold increase in cardiac fibrosis compared with wild-type mice (J:157952)
• aortic root diameter and area are increased compared to in wild-type mice (J:157952)
• aortic root diameter and area are increased compared to in wild-type mice (J:157952)
• some mice exhibit calcification of the ventricular walls, myocardium, and aortic valve unlike wild-type mice (J:157952)
• some mice exhibit calcification of the ventricular walls, myocardium, and aortic valve unlike wild-type mice (J:157952)
• one mouse exhibited unruptured cerebral aneurysms unlike wild-type mice (J:157952)
• one mouse exhibited unruptured cerebral aneurysms unlike wild-type mice (J:157952)
• mice exhibit subarachnoid hemorrhages unlike in wild-type mice (J:157952)
• mice exhibit subarachnoid hemorrhages unlike in wild-type mice (J:157952)
• in some mice (J:157952)
• in some mice (J:157952)

liver/biliary system
• in 5 of 34 mice, likely of cholangiocye origins, and affecting preferentially female mice (J:157952)
• in 5 of 34 mice, likely of cholangiocye origins, and affecting preferentially female mice (J:157952)
• mice exhibit broad band fibrosis along intrahepatic ducts unlike wild-type mice (J:157952)
• hepatic fibrosis is 4- to 5-fold greater than in wild-type mice (J:157952)
• mice exhibit broad band fibrosis along intrahepatic ducts unlike wild-type mice (J:157952)
• hepatic fibrosis is 4- to 5-fold greater than in wild-type mice (J:157952)

nervous system
• mice exhibit subarachnoid hemorrhages unlike in wild-type mice (J:157952)
• mice exhibit subarachnoid hemorrhages unlike in wild-type mice (J:157952)
• the cerebellum is underdeveloped, small in size, or constricted unlike in wild-type mice (J:157952)
• the cerebellum is underdeveloped, small in size, or constricted unlike in wild-type mice (J:157952)
• in some mice (J:157952)
• in some mice (J:157952)
• some mice exhibit ventricular dilation unlike in wild-type mice (J:157952)
• some mice exhibit ventricular dilation unlike in wild-type mice (J:157952)
• the cerebellum is underdeveloped, small in size, or constricted unlike in wild-type mice (J:157952)
• the cerebellum is underdeveloped, small in size, or constricted unlike in wild-type mice (J:157952)

skeleton
• open in some mice (J:157952)
• open in some mice (J:157952)

hematopoietic system
• at 6 months (J:157952)
• at 6 months (J:157952)

homeostasis/metabolism
• at 6 months (J:157952)
• at 6 months (J:157952)
• at 6 months (J:157952)
• at 6 months (J:157952)
• at 5 to 7 months, urine protein levels are decreased compared to in wild-type mice (J:157952)
• at 5 to 7 months, urine protein levels are decreased compared to in wild-type mice (J:157952)
• at 4 months, mice exhibit non-selective proteinuria unlike wild-type mice (J:157952)
• at 4 months, mice exhibit non-selective proteinuria unlike wild-type mice (J:157952)

craniofacial
• open in some mice (J:157952)
• open in some mice (J:157952)

cellular
• proliferation of kidney cells is increased compared to in wild-type mice (J:157952)
• proliferation of kidney cells is increased compared to in wild-type mice (J:157952)

Mouse Models of Human Disease
OMIM ID Ref(s)
Polycystic Kidney Disease, Autosomal Recessive; ARPKD 263200 J:157952


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Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
01/26/2016
MGI 6.02
The Jackson Laboratory