All Phenotypes Tg(Pkhd1-cre)1Igr MGI Mouse

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Phenotypes associated with this allele

Allele Symbol
Allele Name
Allele ID

Tg(Pkhd1-cre)1Igr
transgene insertion 1, Peter Igarashi
MGI:3794109

Summary

6 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
cn1	Pkd2^tm3Som/Pkd2^tm3Som Tg(Pkhd1-cre)1Igr/0	involves: 129S4/SvJae * C57BL/6	MGI:6317329
cn2	Mirc1^tm1.1Tyj/Mirc1^tm1.1Tyj Pkd2^tm3Som/Pkd2^tm3Som Tg(Pkhd1-cre)1Igr/0	involves: 129S4/SvJae * C57BL/6	MGI:6317330
cn3	Hoxb3os^em1Kabo/Hoxb3os^em1Kabo Pkd1^tm2Ggg/Pkd1^tm2Ggg Tg(Pkhd1-cre)1Igr/0	involves: 129S4/SvJae * C57BL/6J	MGI:7612601
cn4	Pkd2^tm3Som/Pkd2^tm3Som Tg(Pkd1)248Som/0 Tg(Pkhd1-cre)1Igr/0	involves: C57BL/6J * SJL/J	MGI:5442314
cn5	Pkd1^tm2Som/Pkd1^tm2Som Tg(Pkhd1-cre)1Igr/0	involves: C57BL/6 * SJL	MGI:3795671
cn6	Pkd2^tm3Som/Pkd2^tm3Som Tg(Pkhd1-cre)1Igr/?	Not Specified	MGI:5430910

Allelic Composition	Pkd2^tm3Som/Pkd2^tm3Som Tg(Pkhd1-cre)1Igr/0
Genetic Background	involves: 129S4/SvJae * C57BL/6

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pkd2^tm3Som mutation (0 available); any Pkd2 mutation (84 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 circulating creatinine level ( J:244067 )

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

Allelic Composition	Mirc1^tm1.1Tyj/Mirc1^tm1.1Tyj Pkd2^tm3Som/Pkd2^tm3Som Tg(Pkhd1-cre)1Igr/0
Genetic Background	involves: 129S4/SvJae * C57BL/6

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mirc1^tm1.1Tyj mutation (1 available); any Mirc1 mutation (6 available)
Pkd2^tm3Som mutation (0 available); any Pkd2 mutation (84 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

Allelic Composition	Hoxb3os^em1Kabo/Hoxb3os^em1Kabo Pkd1^tm2Ggg/Pkd1^tm2Ggg Tg(Pkhd1-cre)1Igr/0
Genetic Background	involves: 129S4/SvJae * C57BL/6J

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Hoxb3os^em1Kabo mutation (0 available); any Hoxb3os mutation (3 available)
Pkd1^tm2Ggg mutation (1 available); any Pkd1 mutation (153 available)
Tg(Pkhd1-cre)1Igr mutation (0 available)

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

renal/urinary system

increased kidney cell proliferation ( J:345935 )

• percentage of Ki67+ cells is increased by 52% in kidneys, indicating increased cell proliferation

kidney cyst ( J:345935 )

• mice exhibit exacerbated cyst growth in kidneys compared to single Pdk1 homozygotes, with a 64% increase in cyst index, 52% increase in cyst number, and 74% increase in kidney weight/body weight ratio

cellular

increased kidney cell proliferation ( J:345935 )

• percentage of Ki67+ cells is increased by 52% in kidneys, indicating increased cell proliferation

growth/size/body

kidney cyst ( J:345935 )

Allelic Composition	Pkd2^tm3Som/Pkd2^tm3Som 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:
Pkd2^tm3Som mutation (0 available); any Pkd2 mutation (84 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

kidney cyst ( J:188763 )

growth/size/body

kidney cyst ( J:188763 )

Allelic Composition	Pkd1^tm2Som/Pkd1^tm2Som Tg(Pkhd1-cre)1Igr/0
Genetic Background	involves: C57BL/6 * SJL

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pkd1^tm2Som mutation (0 available); any Pkd1 mutation (153 available)
Tg(Pkhd1-cre)1Igr mutation (0 available)

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

renal/urinary system

abnormal kidney morphology ( J:135301 )

• at P24, proliferation of the cyst lining cells in the kidney is increased compared to in wild-type mice

kidney cyst ( J:135301 )

growth/size/body

kidney cyst ( J:135301 )

Allelic Composition	Pkd2^tm3Som/Pkd2^tm3Som Tg(Pkhd1-cre)1Igr/?
Genetic Background	Not Specified

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pkd2^tm3Som mutation (0 available); any Pkd2 mutation (84 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)
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