Phenotypes associated with this allele

• Allele Symbol: Pkd2^tm2Som
• Allele Name: targeted mutation 2, Stefan Somlo
• Allele ID: MGI:1860866
• Summary: 15 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
hm1	Pkd2^tm2Som/Pkd2^tm2Som	involves: 129/Sv * 129S2/SvPas * C57BL/6 * SJL	MGI:4440916
hm2	Pkd2^tm2Som/Pkd2^tm2Som	involves: 129/Sv * C57BL/6J * SJL	MGI:2174705
ht3	Pkd2^tm2Som/Pkd2^+	involves: 129/Sv * C57BL/6J * SJL	MGI:2174706
ht4	Pkd2^tm2Som/Pkd2^+	involves: 129/Sv * C57BL/6 * SJL	MGI:3795606
ht5	Pkd2^tm1Som/Pkd2^tm2Som	involves: 129/Sv * C57BL/6J * SJL	MGI:3617451
cn6	Pkd2^tm2Som/Pkd2^+ Prkcsh^tm1Som/Prkcsh^tm1Som Tg(Cdh16-cre)91Igr/0	involves: 129 * C57BL/6 * C57BL/6J * ICR * SJL	MGI:5442313
cn7	Pkd2^tm2Som/Pkd2^+ Sec63^tm1Som/Sec63^tm1Som Tg(Cdh16-cre)91Igr/0	involves: 129 * C57BL/6 * C57BL/6J * ICR * SJL	MGI:5442322
cx8	Pde1a^em1Cjwa/Pde1a^em1Cjwa Pkd2^tm1Som/Pkd2^tm2Som	involves: 129 * C57BL/6J * C57BL/6N * SJL	MGI:6711716
cx9	Pkd1^tm1.1Pcha/Pkd1^tm1.1Pcha Pkd2^tm2Som/Pkd2^+	involves: 129 * C57BL/6J * SJL	MGI:5697042
cx10	Pkd1^tm1.1Pcha/Pkd1^tm1.1Pcha Pkd2^tm1Som/Pkd2^tm2Som	involves: 129 * C57BL/6J * SJL	MGI:5697045
cx11	Pkd1^tm1.1Pcha/Pkd1^+ Pkd2^tm1Som/Pkd2^tm2Som	involves: 129 * C57BL/6J * SJL	MGI:5697046
cx12	Hdac5^tm1Eno/Hdac5^+ Pkd2^tm2Som/Pkd2^tm2Som	involves: 129/Sv * 129S2/SvPas * C57BL/6 * SJL	MGI:4440917
cx13	Hdac5^tm1Eno/Hdac5^tm1Eno Pkd2^tm2Som/Pkd2^tm2Som	involves: 129/Sv * 129S2/SvPas * C57BL/6 * SJL	MGI:4440913
cx14	Pkd2^tm2Som/Pkd2^+ Pkhd1^tm1Gwu/Pkhd1^tm1Gwu	involves: 129/Sv * C57BL/6J * SJL	MGI:3826787
cx15	Pkd1^tm1Som/Pkd1^+ Pkd2^tm2Som/Pkd2^+	involves: 129/Sv * C57BL/6 * SJL	MGI:3795605

Genotype
MGI:4440916

hm1

• Allelic Composition: Pkd2^tm2Som/Pkd2^tm2Som
• Genetic Background: involves: 129/Sv * 129S2/SvPas * C57BL/6 * SJL

• ♀: phenotype observed in females
• ♂: phenotype observed in males
• N: normal phenotype

mortality/aging

lethality throughout fetal growth and development, complete penetrance ( J:158762 )

• no mice are present at E18.5

renal/urinary system

kidney cyst ( J:158762 )

• mice develop kidney cysts with flat lining cells unlike wild-type mice

• however, trichostatin A injection reduces kidney formation

growth/size/body

kidney cyst ( J:158762 )

• mice develop kidney cysts with flat lining cells unlike wild-type mice

• however, trichostatin A injection reduces kidney formation

Genotype
MGI:2174705

hm2

• Allelic Composition: Pkd2^tm2Som/Pkd2^tm2Som
• Genetic Background: involves: 129/Sv * C57BL/6J * SJL

mortality/aging

lethality throughout fetal growth and development, complete penetrance ( J:59314 )

• die in utero between E13.5 and parturition, with most undergoing intrauterine demise with autolysis at E16.5

renal/urinary system

kidney cortex cyst ( J:59314 )

• mice exhibit progressive cyst formation beginning at E15.5, with a preponderance of glomerular cysts and fewer tubular cysts

• however, no liver cysts are formed

cardiovascular system

abnormal placental labyrinth vasculature morphology ( J:104483 )

• labyrinth layer has fewer fetal vessels, which form disorganized networks with reduced branching

atrial septal defect ( J:59314 )

• atrial septum defects are common at E13.5 or later

atrioventricular septal defect ( J:59314 )

• balanced ventricular and atrial septal defects result in an atrioventricular canal defect

ventricular septal defect ( J:59314 )

• defective formation of the interventricular septum at E13.5 or later

pericardial effusion ( J:59314 )

hemorrhage ( J:59314 )

• 50% of E13.5 or later embryos exhibit focal hemorrhages

endocrine/exocrine glands

pancreas cyst ( J:59314 )

• progressive cyst formation in pancreatic ducts with preserved acinar structure beginning at E14.5

homeostasis/metabolism

pericardial effusion ( J:59314 )

abnormal amniotic fluid composition ( J:104483 )

• total protein levels in the amniotic fluid average 2.8-fold higher than in wild-type

hydrops fetalis ( J:59314 )

• 50% of E13.5 or later embryos exhibit progressive total body edema

polyhydramnios ( J:104483 )

• total amniotic fluid volume averages 3.7-fold higher than in controls

embryo

abnormal placental labyrinth vasculature morphology ( J:104483 )

• labyrinth layer has fewer fetal vessels, which form disorganized networks with reduced branching

abnormal primitive node morphology ( J:107740 )

• at E7.5 asymmetric calcium signals are absent in 3 of 3 homozygotes

growth/size/body

pancreas cyst ( J:59314 )

• progressive cyst formation in pancreatic ducts with preserved acinar structure beginning at E14.5

kidney cortex cyst ( J:59314 )

• mice exhibit progressive cyst formation beginning at E15.5, with a preponderance of glomerular cysts and fewer tubular cysts

• however, no liver cysts are formed

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
polycystic kidney disease 2		DOID:0110859	OMIM:613095	J:59314

Genotype
MGI:2174706

ht3

• Allelic Composition: Pkd2^tm2Som/Pkd2⁺
• Genetic Background: involves: 129/Sv * C57BL/6J * SJL

mortality/aging

premature death ( J:59314 )

renal/urinary system

kidney cyst ( J:47035 , J:59314 )

• 21% exhibit unilateral renal cysts (J:47035)

• 29% of 62-88 week old heterozygotes have sparse unilateral renal cysts, however none have bilateral cysts or elevated serum urea nitrogen (J:59314)

liver/biliary system

liver cyst ( J:47035 , J:59314 )

• 10% exhibit liver cysts (J:47035)

• 53% of 62-88 week old heterozygotes have liver cysts (J:59314)

growth/size/body

kidney cyst ( J:47035 , J:59314 )

• 21% exhibit unilateral renal cysts (J:47035)

• 29% of 62-88 week old heterozygotes have sparse unilateral renal cysts, however none have bilateral cysts or elevated serum urea nitrogen (J:59314)

liver cyst ( J:47035 , J:59314 )

• 10% exhibit liver cysts (J:47035)

• 53% of 62-88 week old heterozygotes have liver cysts (J:59314)

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
polycystic kidney disease 2		DOID:0110859	OMIM:613095	J:59314

Genotype
MGI:3795606

ht4

• Allelic Composition: Pkd2^tm2Som/Pkd2⁺
• Genetic Background: involves: 129/Sv * C57BL/6 * SJL

liver/biliary system

liver cyst ( J:78422 )

• at 9 and 12 months of age, 20% and 29%, respectively, of livers exhibit cysts

growth/size/body

liver cyst ( J:78422 )

• at 9 and 12 months of age, 20% and 29%, respectively, of livers exhibit cysts

Genotype
MGI:3617451

ht5

• Allelic Composition: Pkd2^tm1Som/Pkd2^tm2Som
• Genetic Background: involves: 129/Sv * C57BL/6J * SJL

mortality/aging

premature death ( J:59314 )

• median survival time is 65 weeks compared to 94.1 weeks for wild-type

renal/urinary system

increased kidney apoptosis ( J:158354 )

• in cystic renal epithelia cells, apoptotic indices are gradually increased from ~13.55% at 5-8 months of age to ~21.02% at 9-12-months of age, unlike in wild-type cells (~2.86%)

increased kidney cell proliferation ( J:158354 )

• at 5-8 months of age, ~2.86% nuclei of cystic renal epithelial cells are PCNA-positive relative to ~0.85% in wild-type cells; like in hepatic cysts, the % of PCNA-positive nuclei does not change significantly (~2.55%) with age

polycystic kidney ( J:47035 , J:59314 , J:158354 , J:220561 )

• all have bilateral renal cysts (J:47035)

• exhibit renal cyst formation by 6-10 weeks of age that progresses to cystic replacement of normal renal parenchyma later in life (J:59314)

• numerous macroscopically visible cysts are scattered on the kidney surface at an early disease stage (J:158354)

• total renal cystic volume decreases from ~ 40.67% of total kidney parenchyma at 5-8 months to ~31.04% at 9-12 months of age (J:158354)

• primary cilia in cystic renal epithelial cells remain normal despite a tendency to be shorter (J:158354)

• no significant fibrosis is noted around renal cysts in either age group (J:158354)

• mice show a similar level of polycystic kidney disease as single Pkd1^tm1.1Pcha homozygotes (J:220561)

enlarged kidney ( J:158354 )

• mutant kidneys are enlarged with numerous macroscopically visible cysts

increased kidney weight ( J:158354 , J:220561 )

• at 5-8 months of age, mutant kidney weights represent ~2% of total body weight (J:158354)

• by 9-12 months of age, mutant kidney weights account for ~2.6% of total body weight (J:158354)

• kidney weight to body weight ratio is increased to the same extent as in single Pkd1^tm1.1Pcha homozygotes (J:220561)

kidney failure ( J:59314 )

liver/biliary system

increased cholangiocyte apoptosis ( J:158354 )

• at 5-8 months of age, apoptotic indices in cystic cholangiocytes are increased to ~3.41% relative to ~0.56% in wild-type cholangiocytes, but do not change significantly with age (~3.86% at 9-12 months)

abnormal cholangiocyte morphology ( J:158354 )

• cystic cholangiocytes lining intrahepatic bile ducts are enlarged with a thickened basement membrane and a significantly larger width (i.e. distance between two cell junctions) relative to wild-type cholangiocytes

• most cystic cholangiocytes are squamous in appearance

abnormal cholangiocyte primary cilium morphology ( J:158354 )

• cystic cholangiocyte cilia are ~2-fold shorter than wild-type

• ~25% of primary cilia in cystic cholangiocytes appear malformed (e.g. bulbs at the ciliary tip)

bile duct proliferation ( J:47035 , J:158354 )

• 3 of 6 have bile duct proliferation (J:47035)

• at 5-8 months of age, ~18.77% nuclei of cystic cholangiocytes are PCNA-positive relative to ~1.12% in wild-type cholangiocytes; however, the % of PCNA-positive nuclei does not change significantly with age (~20.26% at 9-12 months) (J:158354)

liver cyst ( J:47035 , J:158354 )

• all have 1-10 liver cysts (J:47035)

• at 9-12 months of age, multiple fluid-filled cysts of different size and shapes are noted in the liver parenchyma (J:158354)

• hepatic cysts posses microvilli and have well-developed cellular junctions (J:158354)

• epithelial cells lining liver cysts stain positively for CK-19, suggesting that hepatic cysts arise from cholangiocytes (J:158354)

• most hepatic cysts are lined by a single layer of cholangiocytes while ~10% of liver cysts are multilayered (J:158354)

• total liver cystic volume increases from ~15.84% of total liver parenchyma at 5-8 months to ~23.26% at 9-12 months of age, as assessed by micro-CT scanning (J:158354)

enlarged liver ( J:158354 )

• mutant livers are significantly enlarged with multiple macroscopically visible cysts, often occupying most of the abdominal cavity

increased liver weight ( J:158354 )

• at 5-8 months of age, mutant liver weights represent ~6% of total body weight relative to ~4.7% in wild-type controls

• by 9-12 months of age, mutant liver weights account for ~8% of total body weight, with no significant differences between male and female mice

liver fibrosis ( J:158354 )

• at 5-8 months of age, mild fibrotic deposits are observed around liver cysts, occupying ~2.71% of total liver parenchyma relative to ~0.92% in wild-type controls

• no changes in hepatic fibrosis are found during disease progression

homeostasis/metabolism

increased blood urea nitrogen level ( J:59314 , J:220561 )

• develop azotemia/uremia (J:59314)

• blood urea nitrogen levels are increased to the same extent as in single Pkd1^tm1.1Pcha homozygotes (J:220561)

endocrine/exocrine glands

abnormal cholangiocyte morphology ( J:158354 )

• cystic cholangiocytes lining intrahepatic bile ducts are enlarged with a thickened basement membrane and a significantly larger width (i.e. distance between two cell junctions) relative to wild-type cholangiocytes

• most cystic cholangiocytes are squamous in appearance

abnormal cholangiocyte primary cilium morphology ( J:158354 )

• cystic cholangiocyte cilia are ~2-fold shorter than wild-type

• ~25% of primary cilia in cystic cholangiocytes appear malformed (e.g. bulbs at the ciliary tip)

bile duct proliferation ( J:47035 , J:158354 )

• 3 of 6 have bile duct proliferation (J:47035)

• at 5-8 months of age, ~18.77% nuclei of cystic cholangiocytes are PCNA-positive relative to ~1.12% in wild-type cholangiocytes; however, the % of PCNA-positive nuclei does not change significantly with age (~20.26% at 9-12 months) (J:158354)

pancreas cyst ( J:59314 )

• 50% of mutants over 3 months of age have visible cysts

cellular

abnormal cholangiocyte primary cilium morphology ( J:158354 )

• cystic cholangiocyte cilia are ~2-fold shorter than wild-type

• ~25% of primary cilia in cystic cholangiocytes appear malformed (e.g. bulbs at the ciliary tip)

increased kidney apoptosis ( J:158354 )

• in cystic renal epithelia cells, apoptotic indices are gradually increased from ~13.55% at 5-8 months of age to ~21.02% at 9-12-months of age, unlike in wild-type cells (~2.86%)

increased cholangiocyte apoptosis ( J:158354 )

• at 5-8 months of age, apoptotic indices in cystic cholangiocytes are increased to ~3.41% relative to ~0.56% in wild-type cholangiocytes, but do not change significantly with age (~3.86% at 9-12 months)

increased kidney cell proliferation ( J:158354 )

• at 5-8 months of age, ~2.86% nuclei of cystic renal epithelial cells are PCNA-positive relative to ~0.85% in wild-type cells; like in hepatic cysts, the % of PCNA-positive nuclei does not change significantly (~2.55%) with age

growth/size/body

pancreas cyst ( J:59314 )

• 50% of mutants over 3 months of age have visible cysts

polycystic kidney ( J:47035 , J:59314 , J:158354 , J:220561 )

• all have bilateral renal cysts (J:47035)

• exhibit renal cyst formation by 6-10 weeks of age that progresses to cystic replacement of normal renal parenchyma later in life (J:59314)

• numerous macroscopically visible cysts are scattered on the kidney surface at an early disease stage (J:158354)

• total renal cystic volume decreases from ~ 40.67% of total kidney parenchyma at 5-8 months to ~31.04% at 9-12 months of age (J:158354)

• primary cilia in cystic renal epithelial cells remain normal despite a tendency to be shorter (J:158354)

• no significant fibrosis is noted around renal cysts in either age group (J:158354)

• mice show a similar level of polycystic kidney disease as single Pkd1^tm1.1Pcha homozygotes (J:220561)

liver cyst ( J:47035 , J:158354 )

• all have 1-10 liver cysts (J:47035)

• at 9-12 months of age, multiple fluid-filled cysts of different size and shapes are noted in the liver parenchyma (J:158354)

• hepatic cysts posses microvilli and have well-developed cellular junctions (J:158354)

• epithelial cells lining liver cysts stain positively for CK-19, suggesting that hepatic cysts arise from cholangiocytes (J:158354)

• most hepatic cysts are lined by a single layer of cholangiocytes while ~10% of liver cysts are multilayered (J:158354)

• total liver cystic volume increases from ~15.84% of total liver parenchyma at 5-8 months to ~23.26% at 9-12 months of age, as assessed by micro-CT scanning (J:158354)

enlarged kidney ( J:158354 )

• mutant kidneys are enlarged with numerous macroscopically visible cysts

increased kidney weight ( J:158354 , J:220561 )

• at 5-8 months of age, mutant kidney weights represent ~2% of total body weight (J:158354)

• by 9-12 months of age, mutant kidney weights account for ~2.6% of total body weight (J:158354)

• kidney weight to body weight ratio is increased to the same extent as in single Pkd1^tm1.1Pcha homozygotes (J:220561)

enlarged liver ( J:158354 )

• mutant livers are significantly enlarged with multiple macroscopically visible cysts, often occupying most of the abdominal cavity

increased liver weight ( J:158354 )

• at 5-8 months of age, mutant liver weights represent ~6% of total body weight relative to ~4.7% in wild-type controls

• by 9-12 months of age, mutant liver weights account for ~8% of total body weight, with no significant differences between male and female mice

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
polycystic kidney disease 2		DOID:0110859	OMIM:613095	J:47035

Genotype
MGI:5442313

cn6

• Allelic Composition: Pkd2^tm2Som/Pkd2⁺; Prkcsh^tm1Som/Prkcsh^tm1Som; Tg(Cdh16-cre)91Igr/0
• Genetic Background: involves: 129 * C57BL/6 * C57BL/6J * ICR * SJL

renal/urinary system

kidney cyst ( J:188763 )

• more severe than in Prkcsh^tm1Som/Prkcsh^tm1Som Tg(Cdh16-cre)91Igr mice

increased kidney weight ( J:188763 )

homeostasis/metabolism

increased blood urea nitrogen level ( J:188763 )

growth/size/body

kidney cyst ( J:188763 )

• more severe than in Prkcsh^tm1Som/Prkcsh^tm1Som Tg(Cdh16-cre)91Igr mice

increased kidney weight ( J:188763 )

Genotype
MGI:5442322

cn7

• Allelic Composition: Pkd2^tm2Som/Pkd2⁺; Sec63^tm1Som/Sec63^tm1Som; Tg(Cdh16-cre)91Igr/0
• Genetic Background: involves: 129 * C57BL/6 * C57BL/6J * ICR * SJL

renal/urinary system

kidney cyst ( J:188763 )

homeostasis/metabolism

increased blood urea nitrogen level ( J:188763 )

growth/size/body

kidney cyst ( J:188763 )

Genotype
MGI:6711716

cx8

• Allelic Composition: Pde1a^em1Cjwa/Pde1a^em1Cjwa; Pkd2^tm1Som/Pkd2^tm2Som
• Genetic Background: involves: 129 * C57BL/6J * C57BL/6N * SJL

renal/urinary system

kidney cyst ( J:246596 )

• administration of desmopressin aggravates the renal cystic disease seen in Pkd2^tm1Som/Pkd2^tm2Som mice

growth/size/body

kidney cyst ( J:246596 )

• administration of desmopressin aggravates the renal cystic disease seen in Pkd2^tm1Som/Pkd2^tm2Som mice

Genotype
MGI:5697042

cx9

• Allelic Composition: Pkd1^tm1.1Pcha/Pkd1^tm1.1Pcha; Pkd2^tm2Som/Pkd2⁺
• Genetic Background: involves: 129 * C57BL/6J * SJL

renal/urinary system

polycystic kidney ( J:220561 )

• mice show an increase in polycystic kidney disease severity than either single mutant

increased kidney weight ( J:220561 )

• kidney weight to body weight ratio is increased compared to Pkd1^tm1.1Pcha homozygotes

homeostasis/metabolism

increased blood urea nitrogen level ( J:220561 )

• blood urea nitrogen levels are increased to the same extent as in single Pkd1^tm1.1Pcha homozygotes

growth/size/body

polycystic kidney ( J:220561 )

• mice show an increase in polycystic kidney disease severity than either single mutant

increased kidney weight ( J:220561 )

• kidney weight to body weight ratio is increased compared to Pkd1^tm1.1Pcha homozygotes

Genotype
MGI:5697045

cx10

• Allelic Composition: Pkd1^tm1.1Pcha/Pkd1^tm1.1Pcha; Pkd2^tm1Som/Pkd2^tm2Som
• Genetic Background: involves: 129 * C57BL/6J * SJL

mortality/aging

premature death ( J:220561 )

• 5 of 10 mice die before 4 months of age

renal/urinary system

polycystic kidney ( J:220561 )

• mice develop rapidly progressive polycystic kidney disease, significantly more severe than other genotypes studied

increased kidney weight ( J:220561 )

• large increase in kidney weight to body weight ratio

homeostasis/metabolism

increased blood urea nitrogen level ( J:220561 )

• large increase in blood urea nitrogen levels

growth/size/body

polycystic kidney ( J:220561 )

• mice develop rapidly progressive polycystic kidney disease, significantly more severe than other genotypes studied

increased kidney weight ( J:220561 )

• large increase in kidney weight to body weight ratio

Genotype
MGI:5697046

cx11

• Allelic Composition: Pkd1^tm1.1Pcha/Pkd1⁺; Pkd2^tm1Som/Pkd2^tm2Som
• Genetic Background: involves: 129 * C57BL/6J * SJL

renal/urinary system

polycystic kidney ( J:220561 )

• mice show a similar level of polycystic kidney disease as single Pkd1^tm1.1Pcha homozygotes

increased kidney weight ( J:220561 )

• kidney weight to body weight ratio is increased to the same extent as in single Pkd1^tm1.1Pcha homozygotes

homeostasis/metabolism

increased blood urea nitrogen level ( J:220561 )

• blood urea nitrogen levels are increased to the same extent as in single Pkd1^tm1.1Pcha homozygotes

growth/size/body

polycystic kidney ( J:220561 )

• mice show a similar level of polycystic kidney disease as single Pkd1^tm1.1Pcha homozygotes

increased kidney weight ( J:220561 )

• kidney weight to body weight ratio is increased to the same extent as in single Pkd1^tm1.1Pcha homozygotes

Genotype
MGI:4440917

cx12

• Allelic Composition: Hdac5^tm1Eno/Hdac5⁺; Pkd2^tm2Som/Pkd2^tm2Som
• Genetic Background: involves: 129/Sv * 129S2/SvPas * C57BL/6 * SJL

renal/urinary system

kidney cyst ( J:158762 )

• mice exhibit reduced kidney cyst formation compared with Pkd2^tm2Som homozygotes

growth/size/body

kidney cyst ( J:158762 )

• mice exhibit reduced kidney cyst formation compared with Pkd2^tm2Som homozygotes

Genotype
MGI:4440913

cx13

• Allelic Composition: Hdac5^tm1Eno/Hdac5^tm1Eno; Pkd2^tm2Som/Pkd2^tm2Som
• Genetic Background: involves: 129/Sv * 129S2/SvPas * C57BL/6 * SJL

mortality/aging

lethality throughout fetal growth and development, complete penetrance ( J:158762 )

• only 1 mouse is present at E18.5

Genotype
MGI:3826787

cx14

• Allelic Composition: Pkd2^tm2Som/Pkd2⁺; Pkhd1^tm1Gwu/Pkhd1^tm1Gwu
• Genetic Background: involves: 129/Sv * C57BL/6J * SJL

renal/urinary system

kidney cyst ( J:143394 )

• at 1 month of age, mice have more sphere-shaped cysts than in Pkdh1^tm1Gwu homozygotes

dilated renal tubule ( J:143394 )

growth/size/body

kidney cyst ( J:143394 )

• at 1 month of age, mice have more sphere-shaped cysts than in Pkdh1^tm1Gwu homozygotes

Genotype
MGI:3795605

cx15

• Allelic Composition: Pkd1^tm1Som/Pkd1⁺; Pkd2^tm2Som/Pkd2⁺
• Genetic Background: involves: 129/Sv * C57BL/6 * SJL

liver/biliary system

liver cyst ( J:78422 )

• at 12 months of age, 43% of livers exhibit cysts

• mice exhibit more cysts than in wild-type mice or either heterozygote alone

renal/urinary system

kidney cyst ( J:78422 )

• at 12 months of age, 29% of kidneys exhibit gross kidney surface cysts with only 1 observed at 9 months of age

• mice exhibit more cysts than in wild-type mice or either heterozygote alone

growth/size/body

kidney cyst ( J:78422 )

• at 12 months of age, 29% of kidneys exhibit gross kidney surface cysts with only 1 observed at 9 months of age

• mice exhibit more cysts than in wild-type mice or either heterozygote alone

liver cyst ( J:78422 )

• at 12 months of age, 43% of livers exhibit cysts

• mice exhibit more cysts than in wild-type mice or either heterozygote alone