About   Help   FAQ
Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID 		Pkd2tm3Som
targeted mutation 3, Stefan Somlo
MGI:5430797
Summary 4 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
cn1
 		Pkd2tm3Som/Pkd2tm3Som
Tg(Pkhd1-cre)1Igr/0 		involves: 129S4/SvJae * C57BL/6 MGI:6317329
cn2
 		Mirc1tm1.1Tyj/Mirc1tm1.1Tyj
Pkd2tm3Som/Pkd2tm3Som
Tg(Pkhd1-cre)1Igr/0 		involves: 129S4/SvJae * C57BL/6 MGI:6317330
cn3
 		Pkd2tm3Som/Pkd2tm3Som
Tg(Pkd1)248Som/0
Tg(Pkhd1-cre)1Igr/0 		involves: C57BL/6J * SJL/J MGI:5442314
cn4
 		Pkd2tm3Som/Pkd2tm3Som
Tg(Pkhd1-cre)1Igr/? 		Not Specified MGI:5430910


Genotype
MGI:6317329
cn1
Allelic
Composition		 Pkd2tm3Som/Pkd2tm3Som
Tg(Pkhd1-cre)1Igr/0
Genetic
Background		 involves: 129S4/SvJae * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pkd2tm3Som mutation (0 available); any Pkd2 mutation (85 available)
Tg(Pkhd1-cre)1Igr mutation (0 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
mortality/aging
premature death ( J:244067 )
• median survival of approximately 70 days

renal/urinary system
increased kidney cell proliferation ( J:244067 )
• increase in cyst epithelial cell proliferation
• mice supplemented with the PPAR-alpha agonist fenofibrate or treated with an anti-miR-17 oligonucleotide show reduced cyst epithelial cell proliferation
polycystic kidney ( J:244067 )
• mice exhibit less aggressive polycystic kidney disease than Pkd1 mutants
• mice supplemented with the PPAR-alpha agonist fenofibrate show reduced cyst index
enlarged kidney ( J:244067 )
• kidney is enlarged due to cysts
• mice supplemented with the PPAR-alpha agonist fenofibrate or treated with an anti-miR-17 oligonucleotide show reduced kidney weight-to-body weight ratios

cellular
increased kidney cell proliferation ( J:244067 )
• increase in cyst epithelial cell proliferation
• mice supplemented with the PPAR-alpha agonist fenofibrate or treated with an anti-miR-17 oligonucleotide show reduced cyst epithelial cell proliferation

homeostasis/metabolism
increased blood urea nitrogen level ( J:244067 )
• BUN levels are increased
• mice treated with an anti-miR-17 oligonucleotide show reduced BUN levels

growth/size/body
polycystic kidney ( J:244067 )
• mice exhibit less aggressive polycystic kidney disease than Pkd1 mutants
• mice supplemented with the PPAR-alpha agonist fenofibrate show reduced cyst index
enlarged kidney ( J:244067 )
• kidney is enlarged due to cysts
• mice supplemented with the PPAR-alpha agonist fenofibrate or treated with an anti-miR-17 oligonucleotide show reduced kidney weight-to-body weight ratios

Mouse Models of Human Disease
 DO ID OMIM ID(s) Ref(s)
polycystic kidney disease 2 DOID:0110859 OMIM:613095
 J:244067




Genotype
MGI:6317330
cn2
Allelic
Composition		 Mirc1tm1.1Tyj/Mirc1tm1.1Tyj
Pkd2tm3Som/Pkd2tm3Som
Tg(Pkhd1-cre)1Igr/0
Genetic
Background		 involves: 129S4/SvJae * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mirc1tm1.1Tyj mutation (1 available); any Mirc1 mutation (6 available)
Pkd2tm3Som mutation (0 available); any Pkd2 mutation (85 available)
Tg(Pkhd1-cre)1Igr mutation (0 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
mortality/aging
premature death ( J:244067 )
• mice have a median survival of 127 days which is 149% increase in median survival compared to single conditional Pkd2 mutants

renal/urinary system
decreased kidney cell proliferation ( J:244067 )
• mice show a 58.9% decrease in the number of proliferating cyst epithelial cells compared to single conditional Pkd2 mutants
kidney cyst ( J:244067 )
• mice show a 15.8% reduction in cyst index compared to single conditional Pkd2 mutants

cellular
decreased kidney cell proliferation ( J:244067 )
• mice show a 58.9% decrease in the number of proliferating cyst epithelial cells compared to single conditional Pkd2 mutants

homeostasis/metabolism
decreased circulating creatinine level ( J:244067 )
• mice show a 31.8% improvement in serum creatinine levels compared to single conditional Pkd2 mutants

growth/size/body
kidney cyst ( J:244067 )
• mice show a 15.8% reduction in cyst index compared to single conditional Pkd2 mutants




Genotype
MGI:5442314
cn3
Allelic
Composition		 Pkd2tm3Som/Pkd2tm3Som
Tg(Pkd1)248Som/0
Tg(Pkhd1-cre)1Igr/0
Genetic
Background		 involves: C57BL/6J * SJL/J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pkd2tm3Som mutation (0 available); any Pkd2 mutation (85 available)
Tg(Pkd1)248Som mutation (0 available)
Tg(Pkhd1-cre)1Igr mutation (0 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
renal/urinary system

growth/size/body
kidney cyst ( J:188763 )




Genotype
MGI:5430910
cn4
Allelic
Composition		 Pkd2tm3Som/Pkd2tm3Som
Tg(Pkhd1-cre)1Igr/?
Genetic
Background		 Not Specified
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pkd2tm3Som mutation (0 available); any Pkd2 mutation (85 available)
Tg(Pkhd1-cre)1Igr mutation (0 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
cellular
abnormal mitotic spindle morphology ( J:185863 )
• 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

renal/urinary system
dilated kidney collecting duct ( J:185863 )
• cystic dilation of collecting duct segments beginning at 8-10 days of age




Contributing Projects:
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
08/02/2024
MGI 6.24 		The Jackson Laboratory