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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Tg(Tagln-cre)1Her
transgene insertion 1, Joachim Herz
MGI:2446975
Summary 44 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
cn1
Abcc6tm1c(EUCOMM)Wtsi/Abcc6tm1c(EUCOMM)Wtsi
Tg(Tagln-cre)1Her/?
B6.Cg-Abcc6tm1c(EUCOMM)Wtsi Tg(Tagln-cre)1Her MGI:6241522
cn2
Jag1tm1Frad/Jag1tm1Frad
Tg(Tagln-cre)1Her/0
B6.Cg-Jag1tm1Frad Tg(Tagln-cre)1Her MGI:5447165
cn3
Lrp1tm2Her/Lrp1tm2Her
Tg(Tagln-cre)1Her/0
B6.Cg-Lrp1tm2Her Tg(Tagln-cre)1Her MGI:4943740
cn4
Rock2tm1.1Itl/Rock2+
Tg(Tagln-cre)1Her/0
B6.Cg-Rock2tm1.1Itl Tg(Tagln-cre)1Her MGI:5702757
cn5
Atp2b1tm1.1Hiraw/Atp2b1tm1.1Hiraw
Tg(Tagln-cre)1Her/0
B6J.Cg-Atp2b1tm1.1Hiraw Tg(Tagln-cre)1Her MGI:5708674
cn6
Abcc9tm1.1Mcn/Abcc9tm1.1Mcn
Tg(Tagln-cre)1Her/0
involves: 129 * 129S1/Sv * 129X1/SvJ * C57BL/6J MGI:5770475
cn7
Notch2tm3Grid/Notch2tm3.1Grid
Tg(Tagln-cre)1Her/?
involves: 129 * C57BL/6 MGI:3778203
cn8
Gt(ROSA)26Sortm3(NOTCH3*R1031C)Sat/Gt(ROSA)26Sor+
Notch3Gt(PST033)Byg/Notch3Gt(PST033)Byg
Tg(Tagln-cre)1Her/0
involves: 129 * C57BL/6 * SJL MGI:5007810
cn9
Tgfbr2tm1Karl/Tgfbr2tm1Karl
Tg(Tagln-cre)1Her/?
Gt(ROSA)26Sortm1Sor/?
involves: 129 * C57BL/6 * SJL MGI:3767614
cn10
Gt(ROSA)26Sortm1(NOTCH3)Sat/Gt(ROSA)26Sor+
Notch3Gt(PST033)Byg/Notch3Gt(PST033)Byg
Tg(Tagln-cre)1Her/0
involves: 129 * C57BL/6 * SJL MGI:5007818
cn11
Gt(ROSA)26Sortm2(NOTCH3*C455R)Sat/Gt(ROSA)26Sor+
Notch3Gt(PST033)Byg/Notch3Gt(PST033)Byg
Tg(Tagln-cre)1Her/0
involves: 129 * C57BL/6 * SJL MGI:5007812
cn12
Agtr1atm1Unc/Agtr1atm1Unc
Efemp2tm1.1Hiya/Efemp2tm1.2Hiya
Tg(Tagln-cre)1Her/0
involves: 129P2/OlaHsd * 129S6/SvEvTac * C57BL/6 * SJL MGI:5585152
cn13
Gt(ROSA)26Sortm1(NOTCH3)Sat/Gt(ROSA)26Sor+
Notch3Gt(PST033)Byg/Notch3Gt(PST033)Byg
Tg(Tagln-cre)1Her/0
involves: 129P2/OlaHsd * 129S6/SvEvTac * C57BL/6 * SJL MGI:4830996
cn14
Agtr2tm1Tin/Y
Efemp2tm1.1Hiya/Efemp2tm1.2Hiya
Tg(Tagln-cre)1Her/0
involves: 129P2/OlaHsd * 129S6/SvEvTac * C57BL/6 * SJL MGI:5585151
cn15
Tbxa2rtm1Cof/Tbxa2rtm1.1Bhk
Tg(Tagln-cre)1Her/0
involves: 129P2/OlaHsd * 129S/SvEv * C57BL/6 * SJL MGI:5319080
cn16
Smad5tm1Huy/Smad5tm1.1Huy
Tg(Tagln-cre)1Her/0
involves: 129P2/OlaHsd * C57BL/6 * CD-1 * SJL MGI:3710360
cn17
Efemp2tm1.1Tynk/Efemp2tm1.2Tynk
Tg(Tagln-cre)1Her/0
involves: 129P2/OlaHsd * C57BL/6 * SJL MGI:4410315
cn18
Engtm2.1Hma/Engtm2.1Hma
Tg(Tagln-cre)1Her/0
involves: 129P2/OlaHsd * C57BL/6 * SJL MGI:5775294
cn19
Prdm6tm1.1Jrld/Prdm6tm1.2Jrld
Tg(Tagln-cre)1Her/0
involves: 129P2/OlaHsd * C57BL/6 * SJL MGI:5576877
cn20
Npr1tm1Kuhn/Npr1tm1Kuhn
Tg(Tagln-cre)1Her/0
involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * SJL MGI:2652140
cn21
Ptgestm1.1Gaf/Ptgestm1.1Gaf
Tg(Tagln-cre)1Her/0
involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * SJL MGI:5570433
cn22
Jag1tm1Grid/Jag1tm2Grid
Tg(Tagln-cre)1Her/0
involves: 129S1/Sv * C57BL/6 * SJL MGI:4867007
cn23
Ntf3tm1Par/Ntf3tm2Jae
Tg(Tagln-cre)1Her/0
involves: 129S1/Sv * C57BL/6 * SJL MGI:5559203
cn24
Ntf3tm2Jae/Ntf3+
Tg(Tagln-cre)1Her/0
involves: 129S1/Sv * C57BL/6 * SJL MGI:5559202
cn25
Pros1tm1Grl/Pros1tm1Grl
Tg(Tagln-cre)1Her/0
involves: 129S1/SvImJ * C57BL/6 * SJL MGI:5499119
cn26
Gt(ROSA)26Sortm3(NOTCH3*R1031C)Sat/Gt(ROSA)26Sor+
Tg(Tagln-cre)1Her/0
involves: 129S2/SvEv * 129S6/SvEvTac * C57BL/6 * SJL MGI:5007819
cn27
Ppargtm2Rev/Ppargtm2Rev
Tg(Tagln-cre)1Her/?
involves: 129S4/SvJae * C57BL/6 * SJL MGI:5444198
cn28
Pdgfrbtm14(Pdgfrb)Sor/Pdgfrb+
Tg(Tagln-cre)1Her/0
involves: 129S4/SvJaeSor * C57BL/6 * SJL MGI:5140930
cn29
Ppp3r1tm2Grc/Ppp3r1tm2Grc
Tg(Tagln-cre)1Her/0
involves: 129S6/SvEvTac * C57BL/6 * SJL MGI:4366031
cn30
Efemp2tm1.1Hiya/Efemp2tm1.2Hiya
Tg(Tagln-cre)1Her/0
involves: 129S6/SvEvTac * C57BL/6 * SJL MGI:4947945
cn31
Hey2tm1Eno/Hey2tm2Eno
Tg(Tagln-cre)1Her/0
involves: 129S6/SvEvTac * C57BL/6 * SJL MGI:3711335
cn32
Lrp1tm2Her/Lrp1tm2Her
Tg(APP695)3Dbo/0
Tg(PSEN1)5Dbo/0
Tg(Tagln-cre)1Her/0
involves: 129S7/SvEvBrd * C3H/HeJ * C57BL/6 * C57BL/6J * SJL MGI:5471581
cn33
Rxfp2tm1Aia/Rxfp2tm1c(EUCOMM)Wtsi
Tg(Tagln-cre)1Her/0
involves: 129S7/SvEvBrd * C57BL/6 * C57BL/6N * SJL MGI:5473320
cn34
Agtr1atm1Uky/Agtr1atm1Uky
Ldlrtm1Her/Ldlrtm1Her
Tg(Tagln-cre)1Her/0
involves: 129S7/SvEvBrd * C57BL/6 * C57BL/6N * SJL MGI:4946109
cn35
Ldlrtm1Her/Ldlrtm1Her
Lrp1tm2Her/Lrp1tm2Her
Tg(Tagln-cre)1Her/0
involves: 129S7/SvEvBrd * C57BL/6J * SJL MGI:4943557
cn36
Lrp1tm2Her/Lrp1tm2Her
Tg(Tagln-cre)1Her/0
involves: 129S7/SvEvBrd * C57BL/6 * SJL MGI:6102946
cn37
Rock2tm1.1Itl/Rock2tm1.1Itl
Tg(Tagln-cre)1Her/0
involves: 129S/SvEv * C57BL/6 * C57BL/6J * SJL MGI:5702755
cn38
Atp1a2tm4.1Ling/Atp1a2tm4.1Ling
Tg(Tagln-cre)1Her/0
involves: Black Swiss * C57BL/6 * SJL MGI:5296817
cn39
Tg(CMV-cat,-ROCK2*)3-1Koba/0
Tg(Tagln-cre)1Her/0
involves: C57BL/6 * C57BL/6JJcl * DBA/2 * SJL MGI:5910538
cn40
Commd9tm1c(KOMP)Wtsi/Commd9tm1c(KOMP)Wtsi
Tg(Tagln-cre)1Her/0
involves: C57BL/6 * C57BL/6N * SJL MGI:5796351
cn41
Agtr1atm1Uky/Agtr1atm1Uky
Tg(Tagln-cre)1Her/0
involves: C57BL/6 * C57BL/6N * SJL MGI:4946108
cn42
Sec24ctm1c(EUCOMM)Wtsi/Sec24ctm1c(EUCOMM)Wtsi
Tg(Tagln-cre)1Her/0
involves: C57BL/6 * SJL MGI:5896388
cn43
Efemp2tm1.1Tynk/Efemp2tm1.1Tynk
Tg(Tagln-cre)1Her/0
involves: C57BL/6 * SJL MGI:4410314
cn44
Gata5tm1.1Nemr/Gata5tm1.1Nemr
Tg(Tagln-cre)1Her/0
involves: C57BL/6 * SJL MGI:5707856


Genotype
MGI:6241522
cn1
Allelic
Composition
Abcc6tm1c(EUCOMM)Wtsi/Abcc6tm1c(EUCOMM)Wtsi
Tg(Tagln-cre)1Her/?
Genetic
Background
B6.Cg-Abcc6tm1c(EUCOMM)Wtsi Tg(Tagln-cre)1Her
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Abcc6tm1c(EUCOMM)Wtsi mutation (1 available); any Abcc6 mutation (47 available)
Tg(Tagln-cre)1Her mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
integument
N
• no calcification occurs in the fibrous capsule surrounding the muzzle vibrissae




Genotype
MGI:5447165
cn2
Allelic
Composition
Jag1tm1Frad/Jag1tm1Frad
Tg(Tagln-cre)1Her/0
Genetic
Background
B6.Cg-Jag1tm1Frad Tg(Tagln-cre)1Her
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Jag1tm1Frad mutation (0 available); any Jag1 mutation (11 available)
Tg(Tagln-cre)1Her mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• most mice die within 2 days of birth with few surviving beyond 4 weeks
• most mice die within 2 days of birth with few surviving beyond 4 weeks

cellular

liver/biliary system
• multifoci
• absent or incompletely formed bile ducts near the portal veins
• biliary epithelial cells are unable to organize into tubules; portal vein mesenchyme is reduced
• epithelial cells fail to aggregate
• accumulation of bile in liver parenchyma
• at birth

homeostasis/metabolism
• total and conjugated

growth/size/body
• at birth and in mice that survive beyond 4 weeks, worsening over time

behavior/neurological
• in mice that survive beyond 4 weeks, worsening over time

cardiovascular system
• multifoci

integument
• in mice that survive beyond 4 weeks, worsening over time

endocrine/exocrine glands
• absent or incompletely formed bile ducts near the portal veins
• biliary epithelial cells are unable to organize into tubules; portal vein mesenchyme is reduced
• epithelial cells fail to aggregate

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
Alagille syndrome DOID:9245 OMIM:118450
OMIM:610205
J:166890




Genotype
MGI:4943740
cn3
Allelic
Composition
Lrp1tm2Her/Lrp1tm2Her
Tg(Tagln-cre)1Her/0
Genetic
Background
B6.Cg-Lrp1tm2Her Tg(Tagln-cre)1Her
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Lrp1tm2Her mutation (1 available); any Lrp1 mutation (26 available)
Tg(Tagln-cre)1Her mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• medial thickness is doubled with an increase in the number of cell nuclei
• increase in the number of disruptions of the elastic layers
• freshly isolated cells are more epithelioid shaped compared to the spindled shaped cells from control mice
• alpha-actin filaments are less abundant and often disrupted in quiescent smooth muscle cells
• aortic smooth muscle cells challenged with PDGF-bb display more rapid alpha-actin filament disassembly and fillapodial extension and enhanced proliferation and migration compared to controls
• cultured aortic smooth muscle cells show enhanced serum induced cell growth
• aortic rings mice fail to reach the 50% level of force generation at any concentration of KCl and phenylephrine tested
• increase in neointimal formation and prolonged neointimal hyperplasia following endothelial denudation of carotid arteries

homeostasis/metabolism
• increase in neointimal formation and prolonged neointimal hyperplasia following endothelial denudation of carotid arteries

muscle
• freshly isolated cells are more epithelioid shaped compared to the spindled shaped cells from control mice
• alpha-actin filaments are less abundant and often disrupted in quiescent smooth muscle cells
• aortic smooth muscle cells challenged with PDGF-bb display more rapid alpha-actin filament disassembly and fillapodial extension and enhanced proliferation and migration compared to controls
• cultured aortic smooth muscle cells show enhanced serum induced cell growth
• aortic rings mice fail to reach the 50% level of force generation at any concentration of KCl and phenylephrine tested




Genotype
MGI:5702757
cn4
Allelic
Composition
Rock2tm1.1Itl/Rock2+
Tg(Tagln-cre)1Her/0
Genetic
Background
B6.Cg-Rock2tm1.1Itl Tg(Tagln-cre)1Her
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Rock2tm1.1Itl mutation (0 available); any Rock2 mutation (10 available)
Tg(Tagln-cre)1Her mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• hypoxia-induced pulmonary hypertension is ameliorated in mutants
• hypoxia-induced increases in right ventricular systolic pressure, right ventricular end-diastolic pressure, peak rates of right ventricular pressure development and relaxation, right ventricle hypertrophy, pulmonary vascular remodeling, the number of CD45+ inflammatory cells in the hypoxic pulmonary artery, and pulmonary vascular cell proliferation are suppressed in mutants compared to controls
• however, mice show no differences in heart rate, systemic blood pressure, or left ventricular hemodynamic parameters before or after chronic hypoxia, no differences in pulmonary vasculature and right ventricular systolic pressure under normoxic conditions, and no differences in hemoglobin levels and platelet counts following long-term exposure to hypoxia compared to controls
• aortic vascular smooth muscle cell proliferation and migration in vitro in response to fetal bovine serum are decreased

immune system
• levels of inflammatory cytokines like interferon-gamma and TNF-alpha secreted by vascular smooth muscle cells are reduced under both normoxic and hypoxic conditions

muscle
• aortic vascular smooth muscle cell proliferation and migration in vitro in response to fetal bovine serum are decreased




Genotype
MGI:5708674
cn5
Allelic
Composition
Atp2b1tm1.1Hiraw/Atp2b1tm1.1Hiraw
Tg(Tagln-cre)1Her/0
Genetic
Background
B6J.Cg-Atp2b1tm1.1Hiraw Tg(Tagln-cre)1Her
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Atp2b1tm1.1Hiraw mutation (0 available); any Atp2b1 mutation (13 available)
Tg(Tagln-cre)1Her mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• at 14 weeks of age, conscious mice show significantly higher mean blood pressure than control mice throughout the day, as measured by 24-hr radiotelemetry
• however, circadian variations in heart rate are not significantly altered
• at 14 weeks of age, conscious mice show significantly higher diastolic blood pressure than control mice throughout the day, as measured by 24-hr radiotelemetry
• under resting conditions, conscious mice exhibit significantly higher systolic blood pressure than control mice at 8 and 22 weeks of age, as measured by the tail-cuff method
• at 14 weeks of age, conscious mice show significantly higher systolic blood pressure than control mice throughout the day, as measured by 24-hr radiotelemetry
• mutant femoral artery rings are hyperreactive to the maximum concentration of phenylephrine (10-5 M) relative to control rings (84.1% vs 54.4% KCl contraction, respectively)

muscle
• mutant femoral artery rings are hyperreactive to the maximum concentration of phenylephrine (10-5 M) relative to control rings (84.1% vs 54.4% KCl contraction, respectively)

homeostasis/metabolism
• in primary culture, mutant aortic vascular smooth muscle cells (VSMCs) show higher intracellular calcium concentrations than control VSMCs both before (baseline) and after phenylephrine stimulation

growth/size/body
N
• mice exhibit normal body weight at 8-36 weeks of age




Genotype
MGI:5770475
cn6
Allelic
Composition
Abcc9tm1.1Mcn/Abcc9tm1.1Mcn
Tg(Tagln-cre)1Her/0
Genetic
Background
involves: 129 * 129S1/Sv * 129X1/SvJ * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Abcc9tm1.1Mcn mutation (0 available); any Abcc9 mutation (4 available)
Tg(Tagln-cre)1Her mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
growth/size/body
• increased heart weight to tibia length ratio relative to wild-type controls

mortality/aging
• mice die within 14 days of birth from cardiomyopathy and heart failure

cardiovascular system
• lipid droplet accumulation in neonatal cardiomyocytes, unlike in wild-type hearts
• increased heart weight to tibia length ratio relative to wild-type controls
• progressive decrease in ejection fraction from P6 to P11
• M-mode echocardiograms revealed a decline in fractional shortening at P11
• however, no increase of apoptosis in P7 ventricles, as shown by TUNEL assays
• pinacidil (a KATP channel opener) fails to produce a robust current increase in neonatal cardiomyocytes, unlike in wild-type controls
• mitochondria from neonatal cardiomyocytes are unresponsive to the KATP agonist diazoxide, consistent with with reduced or absent mitochondrial KATP activity
• rapid loss of mitochondrial membrane potential in isolated neonatal cardiomyocytes after exposure to hydrogen peroxide, indicating reduced resistance to cell stress relative to wild-type cardiomyocytes
• mitochondria show a reduced oxygen consumption rate, lack pinacidil responsiveness, and fail to increase oxygen consumption after FCCP treatment, unlike wild-type mitochondria
• failure to fuse and form larger mitochondria in neonatal cardiomyocytes from P2 to P8
• reduced fatty acid oxidation in neonatal cardiomyocytes after palmitate application
• neonatal cardiomyopathy due to failure of the newborn myocardium to transition normally from fetal to mature (oxidative) metabolism

homeostasis/metabolism
N
• nonfasting serum glucose levels are normal at P5-P7
• reduced fatty acid oxidation in neonatal cardiomyocytes after palmitate application
• cardiac mitochondria show a reduced oxygen consumption rate, lack pinacidil responsiveness, and fail to increase oxygen consumption after treatment with FCCP (a mitochondrial uncoupling agent), unlike wild-type mitochondria
• neonatal cardiomyocytes appear to be in a hypoxic state due to their inability to transition to fatty acid oxidation

cellular
• at P8, cardiac mitochondria display an immature pattern with reduced cross-sectional area and intermitochondrial contacts relative to wild-type mitochondria
• however, mitochondrial DNA content is normal
• failure to fuse and form larger mitochondria in neonatal cardiomyocytes from P2 to P8
• neonatal cardiomyocytes are more susceptible to cell death in response to H2O2-induced stress relative to wild-type controls
• opening of KATP channels with pinacidil is less cardioprotective than in wild-type cardiomyocytes
• reduced fatty acid oxidation in neonatal cardiomyocytes after palmitate application
• mitochondria from neonatal cardiomyocytes are unresponsive to the KATP agonist diazoxide, consistent with with reduced or absent mitochondrial KATP activity
• rapid loss of mitochondrial membrane potential in isolated neonatal cardiomyocytes following cell stress induced by exposure to hydrogen peroxide, unlike in wild-type cardiomyocytes
• mitochondria show a reduced oxygen consumption rate, lack pinacidil responsiveness, and fail to increase oxygen consumption after treatment with FCCP (a mitochondrial uncoupling agent), unlike wild-type mitochondria
• increased reactive oxygen species in isolated neonatal cardiomyocytes, as shown by increased dihydroethidium staining relative to wild-type controls

muscle
• progressive decrease in ejection fraction from P6 to P11
• M-mode echocardiograms revealed a decline in fractional shortening at P11
• however, no increase of apoptosis in P7 ventricles, as shown by TUNEL assays
• neonatal cardiomyopathy due to failure of the newborn myocardium to transition normally from fetal to mature (oxidative) metabolism




Genotype
MGI:3778203
cn7
Allelic
Composition
Notch2tm3Grid/Notch2tm3.1Grid
Tg(Tagln-cre)1Her/?
Genetic
Background
involves: 129 * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Notch2tm3.1Grid mutation (0 available); any Notch2 mutation (63 available)
Notch2tm3Grid mutation (2 available); any Notch2 mutation (63 available)
Tg(Tagln-cre)1Her mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• there is a 50% mortality rate between birth and weaning

cardiovascular system
• pulmonary arteries of E18.5 embryos and newborns are significantly smaller
• aorta diameters of all E18.5 embryos and newborns are decreased compared to wild-type mice
• mean aortic velocity in one month old mice is 1.74 m/s which is significantly faster than the mean velocity of 0.93 m/s for controls
• mean pulmonary velocity in one month old mice is 1.9 m/s which is significantly faster than the mean velocity of 1.5 m/s for controls

homeostasis/metabolism
• is observed in neonates




Genotype
MGI:5007810
cn8
Allelic
Composition
Gt(ROSA)26Sortm3(NOTCH3*R1031C)Sat/Gt(ROSA)26Sor+
Notch3Gt(PST033)Byg/Notch3Gt(PST033)Byg
Tg(Tagln-cre)1Her/0
Genetic
Background
involves: 129 * C57BL/6 * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gt(ROSA)26Sortm3(NOTCH3*R1031C)Sat mutation (1 available); any Gt(ROSA)26Sor mutation (572 available)
Notch3Gt(PST033)Byg mutation (0 available); any Notch3 mutation (7 available)
Tg(Tagln-cre)1Her mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
homeostasis/metabolism
• increased susceptibility to ischemic injury seen in Notch3 null mice is not rescued by expression of the mutant human NOTCH3 at 1 year of age but is rescued at 3 to 6 months of age

nervous system
• increased susceptibility to ischemic injury seen in Notch3 null mice is not rescued by expression of the mutant human NOTCH3 at 1 year of age but is rescued at 3 to 6 months of age

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
CADASIL 1 DOID:0111035 OMIM:125310
J:171887




Genotype
MGI:3767614
cn9
Allelic
Composition
Tgfbr2tm1Karl/Tgfbr2tm1Karl
Tg(Tagln-cre)1Her/?
Gt(ROSA)26Sortm1Sor/?
Genetic
Background
involves: 129 * C57BL/6 * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gt(ROSA)26Sortm1Sor mutation (7 available); any Gt(ROSA)26Sor mutation (572 available)
Tgfbr2tm1Karl mutation (1 available); any Tgfbr2 mutation (27 available)
Tg(Tagln-cre)1Her mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• mice die between E12.5 and E16.5; only resorbed homozygous embryos are recovered at E16.5

embryo
• while yolk sacs are normal at E9.5 by E12.5 yolk sacs are pale, anemic and possess obvious vasculature defects
• at E12.5, yolk sacs are pale and anemic

growth/size/body

cardiovascular system
• while yolk sacs are normal at E9.5 by E12.5 yolk sacs are pale, anemic and possess obvious vasculature defects
• mice exhibit delayed underdeveloped hearts

nervous system
• mice exhibit delayed underdeveloped brains




Genotype
MGI:5007818
cn10
Allelic
Composition
Gt(ROSA)26Sortm1(NOTCH3)Sat/Gt(ROSA)26Sor+
Notch3Gt(PST033)Byg/Notch3Gt(PST033)Byg
Tg(Tagln-cre)1Her/0
Genetic
Background
involves: 129 * C57BL/6 * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gt(ROSA)26Sortm1(NOTCH3)Sat mutation (1 available); any Gt(ROSA)26Sor mutation (572 available)
Notch3Gt(PST033)Byg mutation (0 available); any Notch3 mutation (7 available)
Tg(Tagln-cre)1Her mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
N
• increased susceptibility to ischemic injury seen in Notch3 null mice is rescued by expression of the human NOTCH3




Genotype
MGI:5007812
cn11
Allelic
Composition
Gt(ROSA)26Sortm2(NOTCH3*C455R)Sat/Gt(ROSA)26Sor+
Notch3Gt(PST033)Byg/Notch3Gt(PST033)Byg
Tg(Tagln-cre)1Her/0
Genetic
Background
involves: 129 * C57BL/6 * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gt(ROSA)26Sortm2(NOTCH3*C455R)Sat mutation (1 available); any Gt(ROSA)26Sor mutation (572 available)
Notch3Gt(PST033)Byg mutation (0 available); any Notch3 mutation (7 available)
Tg(Tagln-cre)1Her mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype

Vascular smooth muscle cell abnormalities in Gt(ROSA)26Sortm3(NOTCH3*R1031C)Sat/Gt(ROSA)26Sor+ Tg(Tagln-cre)1Her/0 and Gt(ROSA)26Sortm2(NOTCH3*C455R)Sat/Gt(ROSA)26Sor+ Notch3Gt(PST033)Byg/Notch3Gt(PST033)Byg Tg(Tagln-cre)1Her/0 mice

cardiovascular system
• vascular smooth muscle cell abnormalities with intracellular inclusions are seen at 6 months of age

homeostasis/metabolism
• increased susceptibility to ischemic injury seen in Notch3 null mice is not rescued by expression of the mutant human NOTCH3

muscle
• vascular smooth muscle cell abnormalities with intracellular inclusions are seen at 6 months of age

nervous system
• increased susceptibility to ischemic injury seen in Notch3 null mice is not rescued by expression of the mutant human NOTCH3

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
CADASIL 1 DOID:0111035 OMIM:125310
J:171887




Genotype
MGI:5585152
cn12
Allelic
Composition
Agtr1atm1Unc/Agtr1atm1Unc
Efemp2tm1.1Hiya/Efemp2tm1.2Hiya
Tg(Tagln-cre)1Her/0
Genetic
Background
involves: 129P2/OlaHsd * 129S6/SvEvTac * C57BL/6 * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Agtr1atm1Unc mutation (1 available); any Agtr1a mutation (15 available)
Efemp2tm1.1Hiya mutation (0 available); any Efemp2 mutation (18 available)
Efemp2tm1.2Hiya mutation (0 available); any Efemp2 mutation (18 available)
Tg(Tagln-cre)1Her mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• aortic wall is thicker at 3 months of age compared to single Agtr1a homozygotes




Genotype
MGI:4830996
cn13
Allelic
Composition
Gt(ROSA)26Sortm1(NOTCH3)Sat/Gt(ROSA)26Sor+
Notch3Gt(PST033)Byg/Notch3Gt(PST033)Byg
Tg(Tagln-cre)1Her/0
Genetic
Background
involves: 129P2/OlaHsd * 129S6/SvEvTac * C57BL/6 * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gt(ROSA)26Sortm1(NOTCH3)Sat mutation (1 available); any Gt(ROSA)26Sor mutation (572 available)
Notch3Gt(PST033)Byg mutation (0 available); any Notch3 mutation (7 available)
Tg(Tagln-cre)1Her mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
homeostasis/metabolism
• following filament middle cerebral artery occlusion, mice exhibit reduced infarct volume compared with similarly treated Notch3Gt(PST033)Byg homozygotes

nervous system
• following filament middle cerebral artery occlusion, mice exhibit reduced infarct volume compared with similarly treated Notch3Gt(PST033)Byg homozygotes




Genotype
MGI:5585151
cn14
Allelic
Composition
Agtr2tm1Tin/Y
Efemp2tm1.1Hiya/Efemp2tm1.2Hiya
Tg(Tagln-cre)1Her/0
Genetic
Background
involves: 129P2/OlaHsd * 129S6/SvEvTac * C57BL/6 * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Agtr2tm1Tin mutation (0 available); any Agtr2 mutation (8 available)
Efemp2tm1.1Hiya mutation (0 available); any Efemp2 mutation (18 available)
Efemp2tm1.2Hiya mutation (0 available); any Efemp2 mutation (18 available)
Tg(Tagln-cre)1Her mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• hyperproliferation and disarray of aorta smooth muscle cells at 3 months of age
• the medium of the aortic wall is thicker than in single Agtr2 mutants, with hyperproliferation and disarray of smooth muscle cells at 3 months of age
• mice develop aneurysms
• treatment with losartan completely prevents aneurysms

muscle
• hyperproliferation and disarray of aorta smooth muscle cells at 3 months of age

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
aortic aneurysm DOID:3627 J:213282




Genotype
MGI:5319080
cn15
Allelic
Composition
Tbxa2rtm1Cof/Tbxa2rtm1.1Bhk
Tg(Tagln-cre)1Her/0
Genetic
Background
involves: 129P2/OlaHsd * 129S/SvEv * C57BL/6 * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tbxa2rtm1.1Bhk mutation (1 available); any Tbxa2r mutation (10 available)
Tbxa2rtm1Cof mutation (0 available); any Tbxa2r mutation (10 available)
Tg(Tagln-cre)1Her mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
respiratory system
• mice exposed to increasing doses of thromboxane receptor agonist U-46619 exhibit an attenuated dose-dependent increase in airway resistance unlike control mice
• mice exposed to U-46619 and methacholine exhibit less of an increased in airway resistance compared with control mice
• however, mice exposed ovalbumin and U-46619 exhibit dose-dependent increase in airway resistance




Genotype
MGI:3710360
cn16
Allelic
Composition
Smad5tm1Huy/Smad5tm1.1Huy
Tg(Tagln-cre)1Her/0
Genetic
Background
involves: 129P2/OlaHsd * C57BL/6 * CD-1 * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Smad5tm1.1Huy mutation (0 available); any Smad5 mutation (2 available)
Smad5tm1Huy mutation (0 available); any Smad5 mutation (2 available)
Tg(Tagln-cre)1Her mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• mild dilation of the heart
• no cardiomyopathy, cardiac hypertrophy, or differences in heart rate and have normal blood vessel development, blood vessel morphology in adults, angiogenesis and vascular remodeling response
• at 9 months of age, females, but not males, show larger systolic and diastolic LV internal diameters and decreased fractional shortening, indicating decreased cardiac contractility

homeostasis/metabolism
• females, but not males, perform worse in a treadmill experiment

muscle
• at 9 months of age, females, but not males, show larger systolic and diastolic LV internal diameters and decreased fractional shortening, indicating decreased cardiac contractility

behavior/neurological
• females, but not males, perform worse in a treadmill experiment




Genotype
MGI:4410315
cn17
Allelic
Composition
Efemp2tm1.1Tynk/Efemp2tm1.2Tynk
Tg(Tagln-cre)1Her/0
Genetic
Background
involves: 129P2/OlaHsd * C57BL/6 * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Efemp2tm1.1Tynk mutation (0 available); any Efemp2 mutation (18 available)
Efemp2tm1.2Tynk mutation (0 available); any Efemp2 mutation (18 available)
Tg(Tagln-cre)1Her mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
growth/size/body

muscle

cardiovascular system
• at P0, the aortic laminae is disrupted unlike in wild-type mice
• at P14, the elastic laminae is disrupted unlike in wild-type mice
• at 12 weeks, aorta are less distensible than in wild-type mice
• mice loss the internal elastic lamina in the ascending aorta unlike wild-type mice
• slightly elongated
• the ascending aorta from the aortic root to just distal to the branching point of the brachiocephalic artery is dilated
• however, no aortic stenosis is observed
• severe ascending aortic aneurysms
• at 12 weeks, aorta are less distensible and stiffer than in wild-type mice




Genotype
MGI:5775294
cn18
Allelic
Composition
Engtm2.1Hma/Engtm2.1Hma
Tg(Tagln-cre)1Her/0
Genetic
Background
involves: 129P2/OlaHsd * C57BL/6 * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Engtm2.1Hma mutation (1 available); any Eng mutation (15 available)
Tg(Tagln-cre)1Her mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• dilated and dysmorphic vessels in brains of 5 week old mice
• 90% of 5 week old mice exhibit arteriovenous malformations with greatly enlarged and tortuous vessels in the brain
• more than 80% of mice have one arteriovenous malformation lesion, while the remaining 20% have 2-3 lesions
• arteriovenous (A-V) shunting and hemorrhage are seen in some brain and spinal cord lesions
• arteriovenous malformations are also seen in the spinal cord and intestine
• hemorrhage is seen in some brain and spinal cord lesions
• macrophages and microhemorrhage are seen around dysplastic vessels in the brain

digestive/alimentary system
• arteriovenous malformations are also seen in the spinal cord and intestine

mortality/aging
• more than 50% of mice before 6 weeks of age




Genotype
MGI:5576877
cn19
Allelic
Composition
Prdm6tm1.1Jrld/Prdm6tm1.2Jrld
Tg(Tagln-cre)1Her/0
Genetic
Background
involves: 129P2/OlaHsd * C57BL/6 * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Prdm6tm1.1Jrld mutation (1 available); any Prdm6 mutation (14 available)
Prdm6tm1.2Jrld mutation (0 available); any Prdm6 mutation (14 available)
Tg(Tagln-cre)1Her mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype

Massive hemorrhage in Prdm6tm1.1Jrld/Prdm6tm1.2Jrld; Tg(Tagln-cre)1Her lungs

mortality/aging
• newborn mice die within 2 days after birth
• newborn mice die within 2 days after birth

cardiovascular system
• massive

respiratory system
• massive




Genotype
MGI:2652140
cn20
Allelic
Composition
Npr1tm1Kuhn/Npr1tm1Kuhn
Tg(Tagln-cre)1Her/0
Genetic
Background
involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Npr1tm1Kuhn mutation (0 available); any Npr1 mutation (46 available)
Tg(Tagln-cre)1Her mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• unlike hypertensive homozygous null mice, conscious conditional mutants exhibit normal chronic arterial BPs under resting conditions, despite abolition of vasorelaxing responses to ANP
• however, infusion of ANP at a dose of 500 ng/kg BW fails to lower arterial BP in conscious conditional mutants but significantly reduces systolic and diastolic BP in wild-type and homozygous Npr1tm1Kuhn mice
• notably, acute vascular volume expansion results in a rapid, significant increase in systolic BP and slight increase in diastolic BP during the 15 min of high-volume infusion; such changes are fully reversible 10 min after volume overload and are absent in homozygous Npr1tm1Kuhn mice
• vasorelaxing responses to atrial natriuretic peptide (ANP) are abolished in isolated preconstricted arteries from conditional mutant mice
• small relaxation in response to high ANP concentrations, also observed in phenylephrine (PE)-contracted arteries in the absence of ANP, indicating a spontaneous (ANP-independent) loss of tone

muscle
• vasorelaxing responses to atrial natriuretic peptide (ANP) are abolished in isolated preconstricted arteries from conditional mutant mice
• small relaxation in response to high ANP concentrations, also observed in phenylephrine (PE)-contracted arteries in the absence of ANP, indicating a spontaneous (ANP-independent) loss of tone




Genotype
MGI:5570433
cn21
Allelic
Composition
Ptgestm1.1Gaf/Ptgestm1.1Gaf
Tg(Tagln-cre)1Her/0
Genetic
Background
involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ptgestm1.1Gaf mutation (0 available); any Ptges mutation (43 available)
Tg(Tagln-cre)1Her mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
homeostasis/metabolism
• following wire injury, mice exhibit accelerated intimal hyperplasia with increased intima to media ratio and stenosis percent compared with control mice
• decreased IL1beta-stimulated prostaglandin E2
• increased IL1beta-stimulated prostaglandin D2 and I2 levels

cardiovascular system
N
• whether fed standard chow or a high salt diet, mice exhibit normal modulation of blood pressure and thrombogenesis
• following wire injury, mice exhibit accelerated intimal hyperplasia with increased intima to media ratio and stenosis percent compared with control mice




Genotype
MGI:4867007
cn22
Allelic
Composition
Jag1tm1Grid/Jag1tm2Grid
Tg(Tagln-cre)1Her/0
Genetic
Background
involves: 129S1/Sv * C57BL/6 * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Jag1tm1Grid mutation (1 available); any Jag1 mutation (11 available)
Jag1tm2Grid mutation (2 available); any Jag1 mutation (11 available)
Tg(Tagln-cre)1Her mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• 50% of mutants die by P1
• no mutants survive past P2

cardiovascular system
• 95% of mutants exhibit patent ductus arteriosus
• defects in vascular smooth muscle cell differentiation in the outer layers of the medial wall of the ductus arteriosus and descending aorta at E17.5 when the ductus arteriosus is still patent, however the ascending limb of the aorta and the pulmonary artery trunk have normal vascular smooth muscle cell differentiation

homeostasis/metabolism

muscle
• defects in vascular smooth muscle cell differentiation in the outer layers of the medial wall of the ductus arteriosus and descending aorta at E17.5 when the ductus arteriosus is still patent, however the ascending limb of the aorta and the pulmonary artery trunk have normal vascular smooth muscle cell differentiation

cellular
• 95% of mutants exhibit patent ductus arteriosus




Genotype
MGI:5559203
cn23
Allelic
Composition
Ntf3tm1Par/Ntf3tm2Jae
Tg(Tagln-cre)1Her/0
Genetic
Background
involves: 129S1/Sv * C57BL/6 * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ntf3tm1Par mutation (0 available); any Ntf3 mutation (6 available)
Ntf3tm2Jae mutation (1 available); any Ntf3 mutation (6 available)
Tg(Tagln-cre)1Her mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
behavior/neurological
• mice exhibit increased meal duration with reduced rate of food intake compared with control mice
• mice exhibit a satiation deficit compared with control mice
• however, mice exhibit normal meal size and oropharyngeal-positive feedback signaling

nervous system
• mice exhibit reduced solitary tract nucleus and area postrema meal-induced c-Fos activation, suggesting a decrease in vagal afferent signaling, compared with control mice

growth/size/body
N
• mice exhibit normal body weight




Genotype
MGI:5559202
cn24
Allelic
Composition
Ntf3tm2Jae/Ntf3+
Tg(Tagln-cre)1Her/0
Genetic
Background
involves: 129S1/Sv * C57BL/6 * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ntf3tm2Jae mutation (1 available); any Ntf3 mutation (6 available)
Tg(Tagln-cre)1Her mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
behavior/neurological
• mice exhibit increased meal duration with reduced rate of food intake compared with control mice
• however, meal size is normal




Genotype
MGI:5499119
cn25
Allelic
Composition
Pros1tm1Grl/Pros1tm1Grl
Tg(Tagln-cre)1Her/0
Genetic
Background
involves: 129S1/SvImJ * C57BL/6 * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pros1tm1Grl mutation (1 available); any Pros1 mutation (2 available)
Tg(Tagln-cre)1Her mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system




Genotype
MGI:5007819
cn26
Allelic
Composition
Gt(ROSA)26Sortm3(NOTCH3*R1031C)Sat/Gt(ROSA)26Sor+
Tg(Tagln-cre)1Her/0
Genetic
Background
involves: 129S2/SvEv * 129S6/SvEvTac * C57BL/6 * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gt(ROSA)26Sortm3(NOTCH3*R1031C)Sat mutation (1 available); any Gt(ROSA)26Sor mutation (572 available)
Tg(Tagln-cre)1Her mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype

Vascular smooth muscle cell abnormalities in Gt(ROSA)26Sortm3(NOTCH3*R1031C)Sat/Gt(ROSA)26Sor+ Tg(Tagln-cre)1Her/0 and Gt(ROSA)26Sortm2(NOTCH3*C455R)Sat/Gt(ROSA)26Sor+ Notch3Gt(PST033)Byg/Notch3Gt(PST033)Byg Tg(Tagln-cre)1Her/0 mice

cardiovascular system
• thinning of the vascular smooth muscle layers
• osmiophilic granular deposits in mice older than 12 months of age
• vascular smooth muscle cell abnormalities with intracellular inclusions and lipid droplets that get more severe with age
• thinning of the vascular smooth muscle layers

muscle
• vascular smooth muscle cell abnormalities with intracellular inclusions and lipid droplets that get more severe with age
• thinning of the vascular smooth muscle layers

nervous system
• osmiophilic granular deposits in mice older than 12 months of age

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
CADASIL 1 DOID:0111035 OMIM:125310
J:171887




Genotype
MGI:5444198
cn27
Allelic
Composition
Ppargtm2Rev/Ppargtm2Rev
Tg(Tagln-cre)1Her/?
Genetic
Background
involves: 129S4/SvJae * C57BL/6 * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ppargtm2Rev mutation (1 available); any Pparg mutation (29 available)
Tg(Tagln-cre)1Her mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
growth/size/body

muscle

cardiovascular system
• increased muscularization of pulmonary arteries at the alveolar wall
• elevated right ventricular systolic pressure




Genotype
MGI:5140930
cn28
Allelic
Composition
Pdgfrbtm14(Pdgfrb)Sor/Pdgfrb+
Tg(Tagln-cre)1Her/0
Genetic
Background
involves: 129S4/SvJaeSor * C57BL/6 * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pdgfrbtm14(Pdgfrb)Sor mutation (1 available); any Pdgfrb mutation (59 available)
Tg(Tagln-cre)1Her mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
N
• mice exhibit normal survival

cardiovascular system
• mice exhibit alterations in the ascending aorta at 2 weeks that progresses through the arch and descending aorta to affect the entire thoracic and abdominal aorta by 4 weeks unlike in wild-type mice
• mice exhibit reduced periaortic adipose compared to in wild-type mice
• authors state that mice exhibit the aorta phenotype observed in mice expressing the floxed allele and Tg(Sox2-cre)1Amc
• however, aorta histology is normal in neonates
• increased radius
• 2-fold dilation that reaches homeostasis without rupture or apoptosis of vascular smooth muscle cells
• at P7, retinal capillary size is increased compared to in wild-type mice
• at P12, brain capillary size is increased compared to in wild-type mice
• reduced vessel density
• pericyte coverage of brain and retinal vasculature is increased compared to in wild-type mice
• reduced vessel density
• mice exhibit increased vascular smooth muscle cell cellularity within the aorta compared with wild-type mice
• at E18.5, mice exhibit increased vascular smooth muscle cell proliferation in the aorta compared with wild-type mice
• cultured aorta release 4-fold more vascular smooth muscle cell due to increased proliferation compared with wild-type cultures

adipose tissue
• mice exhibit reduced periaortic adipose compared to in wild-type mice

growth/size/body
N
• mice exhibit normal growth

muscle
• mice exhibit increased vascular smooth muscle cell cellularity within the aorta compared with wild-type mice
• at E18.5, mice exhibit increased vascular smooth muscle cell proliferation in the aorta compared with wild-type mice
• cultured aorta release 4-fold more vascular smooth muscle cell due to increased proliferation compared with wild-type cultures

nervous system
• reduced vessel density

vision/eye
• reduced vessel density




Genotype
MGI:4366031
cn29
Allelic
Composition
Ppp3r1tm2Grc/Ppp3r1tm2Grc
Tg(Tagln-cre)1Her/0
Genetic
Background
involves: 129S6/SvEvTac * C57BL/6 * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ppp3r1tm2Grc mutation (1 available); any Ppp3r1 mutation (27 available)
Tg(Tagln-cre)1Her mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
N
• mice are grossly normal and display normal coronary endothelial patterning at E12.5; mice live to adulthood and are indistinguishable from wild-type littermates




Genotype
MGI:4947945
cn30
Allelic
Composition
Efemp2tm1.1Hiya/Efemp2tm1.2Hiya
Tg(Tagln-cre)1Her/0
Genetic
Background
involves: 129S6/SvEvTac * C57BL/6 * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Efemp2tm1.1Hiya mutation (0 available); any Efemp2 mutation (18 available)
Efemp2tm1.2Hiya mutation (0 available); any Efemp2 mutation (18 available)
Tg(Tagln-cre)1Her mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• mice die at 2 months of age

cardiovascular system
• increase in proliferation of aorta vessel wall at 1 month of age
• vessel area is increased in the aorta
• increased in thickness
• smooth muscle cells of the aorta are abnormal by P14 and their proliferation extends throughout the medial layer
• hyperproliferation and disarray of smooth muscle cells in the ascending aorta wall at 3 months of age
• focal lesions with thickened aortic wall (J:170883)
• increased collagen fibers (J:170883)
• cellularity in the aorta is increased by P7, especially in the subendothelial layer and near the adventitia (J:213282)
• captopril, an ACE inhibitor, or losartan treatment of pregnant females and continued until 3 months of age, completely prevents aneurysm formation and hyperproliferaton and disarray of smooth muscle cells in the aortic wall, however amount of collagen is decreased, and elastic fibers remain irregular (J:213282)
• administration of losartan starting at P7 completely prevents aneurysms, however administration from P30 to P90 does not prevent aneurysms (J:213282)
• however, propranolol, a beta-adrenergic receptor blocker, treatment shows modest inhibitory effects on aneurysm formation (J:213282)
• slight dilatation of the ascending aorta is seen at P14
• vascular smooth muscle cells exhibit defective differentiation compared to in wild-type mice
• pulse pressures are increased 50%
• captopril treatment decreases this increase in pulse pressure
• mice show a trend toward lower average diastolic pressures
• captopril treatment decreases systolic blood pressures about 20% in mutants, however losartan or propranolol have no effect on blood pressure
• compliance of the ascending aorta is decreased 60-80% in the middle-to high-pressure range (75-175 mmHg), indicating that the vessel wall is stiffer
• treatment with losartan or captopril does not completely reverse the increase in vessel wall stiffness

muscle
• vascular smooth muscle cells exhibit defective differentiation compared to in wild-type mice
• smooth muscle cells of the aorta are abnormal by P14 and their proliferation extends throughout the medial layer
• hyperproliferation and disarray of smooth muscle cells in the ascending aorta wall at 3 months of age

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
aortic aneurysm DOID:3627 J:213282




Genotype
MGI:3711335
cn31
Allelic
Composition
Hey2tm1Eno/Hey2tm2Eno
Tg(Tagln-cre)1Her/0
Genetic
Background
involves: 129S6/SvEvTac * C57BL/6 * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Hey2tm1Eno mutation (1 available); any Hey2 mutation (4 available)
Hey2tm2Eno mutation (0 available); any Hey2 mutation (4 available)
Tg(Tagln-cre)1Her mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• mice display partial perinatal lethality

cardiovascular system
• VSDs are observed
• contractile dysfunction observed

muscle
• contractile dysfunction observed




Genotype
MGI:5471581
cn32
Allelic
Composition
Lrp1tm2Her/Lrp1tm2Her
Tg(APP695)3Dbo/0
Tg(PSEN1)5Dbo/0
Tg(Tagln-cre)1Her/0
Genetic
Background
involves: 129S7/SvEvBrd * C3H/HeJ * C57BL/6 * C57BL/6J * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Lrp1tm2Her mutation (1 available); any Lrp1 mutation (26 available)
Tg(APP695)3Dbo mutation (3 available)
Tg(PSEN1)5Dbo mutation (1 available)
Tg(Tagln-cre)1Her mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
• amyloid beta deposition in the brains is higher than in controls with only Tg(PSEN1)5Dbo and Tg(APP69ff5)3Dbo at 13-14 months of age
• amyloid beta deposition is seen in the cortical parenchyma and in cortical vessels as cerebral amyloid angiopathy
• concentrations of insoluble amyloid beta40 and amyloid beta42 is higher than in controls at 13-14 months of age but not at 3 months
• despite the increase in amyloid beta deposition, APP processing and levels of amyloid beta degrading enzymes are normal, indicating that the increase is due to a disturbance of lysosomal-mediated amyloid beta clearance
• amyloid beta deposition is seen in cortical vessels as cerebral amyloid angiopathy
• activation of astrocytes is enhanced compared to controls with only Tg(PSEN1)5Dbo and Tg(APP69ff5)3Dbo at 1 year of age

homeostasis/metabolism
• amyloid beta deposition in the brains is higher than in controls with only Tg(PSEN1)5Dbo and Tg(APP69ff5)3Dbo at 13-14 months of age
• amyloid beta deposition is seen in the cortical parenchyma and in cortical vessels as cerebral amyloid angiopathy
• concentrations of insoluble amyloid beta40 and amyloid beta42 is higher than in controls at 13-14 months of age but not at 3 months
• despite the increase in amyloid beta deposition, APP processing and levels of amyloid beta degrading enzymes are normal, indicating that the increase is due to a disturbance of lysosomal-mediated amyloid beta clearance
• amyloid beta deposition is seen in cortical vessels as cerebral amyloid angiopathy

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
Alzheimer's disease DOID:10652 J:192453
cerebral amyloid angiopathy DOID:9246 J:192453




Genotype
MGI:5473320
cn33
Allelic
Composition
Rxfp2tm1Aia/Rxfp2tm1c(EUCOMM)Wtsi
Tg(Tagln-cre)1Her/0
Genetic
Background
involves: 129S7/SvEvBrd * C57BL/6 * C57BL/6N * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Rxfp2tm1Aia mutation (0 available); any Rxfp2 mutation (31 available)
Rxfp2tm1c(EUCOMM)Wtsi mutation (0 available); any Rxfp2 mutation (31 available)
Tg(Tagln-cre)1Her mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
reproductive system
N
• mice exhibit normal testis position




Genotype
MGI:4946109
cn34
Allelic
Composition
Agtr1atm1Uky/Agtr1atm1Uky
Ldlrtm1Her/Ldlrtm1Her
Tg(Tagln-cre)1Her/0
Genetic
Background
involves: 129S7/SvEvBrd * C57BL/6 * C57BL/6N * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Agtr1atm1Uky mutation (1 available); any Agtr1a mutation (15 available)
Ldlrtm1Her mutation (19 available); any Ldlr mutation (34 available)
Tg(Tagln-cre)1Her mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
N
• mice fed a high-fat diet exhibit the same amount of angiotensin-induced expansion of ascending aortas and aneurysms as in similarly treated Ldlrtm1Her homozygotes




Genotype
MGI:4943557
cn35
Allelic
Composition
Ldlrtm1Her/Ldlrtm1Her
Lrp1tm2Her/Lrp1tm2Her
Tg(Tagln-cre)1Her/0
Genetic
Background
involves: 129S7/SvEvBrd * C57BL/6J * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ldlrtm1Her mutation (19 available); any Ldlr mutation (34 available)
Lrp1tm2Her mutation (1 available); any Lrp1 mutation (26 available)
Tg(Tagln-cre)1Her mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• progressive thickening of the aorta wall with age
• thickening is primarily caused by increased smooth muscle cell proliferation
• aortas are consistently distended and dilated
• pronounced atherosclerosis is seen in the aorta
• on a high cholesterol diet
• addition of Gleevec to the diet protects against atherosclerotic lesion formation
• almost complete occlusion of the mesenteric arteries
• increase in vascular smooth muscle cell proliferation

homeostasis/metabolism
N
• no changes in cholesterol or triglyceride levels are detected compared to mice homozygous for Ldlrtm1Her alone

muscle
• increase in vascular smooth muscle cell proliferation




Genotype
MGI:6102946
cn36
Allelic
Composition
Lrp1tm2Her/Lrp1tm2Her
Tg(Tagln-cre)1Her/0
Genetic
Background
involves: 129S7/SvEvBrd * C57BL/6 * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Lrp1tm2Her mutation (1 available); any Lrp1 mutation (26 available)
Tg(Tagln-cre)1Her mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
growth/size/body
• increase in heart size in mice older than 36 weeks of age
• heart weight to body weight ratios are increased throughout the age range from 16 to 70 weeks of age

cardiovascular system
• ascending aortas are characterized by increased cell nuclei and fractured elastic laminae and show progressive intra-lamellar thickening, extracellular matrix deposition, and cellular disorganization
• medial layers of the ascending aorta exhibits increased cellular and intracellular accumulation of connective tissue growth factor
• dilation of aortic roots is seen beginning at 16 weeks of age and progresses with age
• dilated ascending aortas in mice older than 36 weeks of age
• coronary arteries within the left ventricle show accumulation of collagen within the tunica media and adventitia
• increase in perivascular fibrosis with outward extension of fibrotic lesions surrounding intramural coronary arteries in 48 week old mice
• extensive and continuous fibrotic lesions tracts extending outward from coronary arteries are sporadically seen in ventricle walls
• increase in myocyte longitudinal area in parenchymal regions of the left ventricle free wall in 48 week old mice
• increase in heart size in mice older than 36 weeks of age
• heart weight to body weight ratios are increased throughout the age range from 16 to 70 weeks of age
• left ventricle enlargement in 48 week old mice
• interstitial fibrosis in intramural coronary arteries of left ventricle free wall and extensive fibrosis in left ventricle papillary muscles
• however, no pericellular fibrosis is seen
• a 38% reduction in fractional shortening and 43% reduction in ejection fraction
• echocardiography shows a reduction in systolic function, with a 38% reduction in fractional shortening, 43% reduction in ejection fraction, and 68% increase in left ventricular diameter in 36 week old mice
• mice show age-dependent increase in incidence of aortic insufficiency, with all mice showing it at 30 weeks of age
• 24 week old mice exhibit a 44% higher pulse pressure than controls
• captopril treatment reduces pulse pressure to levels seen in untreated control mice
• 16% reduction in diastolic pressure in 24 week old mice
• however, systolic blood pressure is normal

muscle
• increase in myocyte longitudinal area in parenchymal regions of the left ventricle free wall in 48 week old mice
• a 38% reduction in fractional shortening and 43% reduction in ejection fraction

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
cardiomyopathy DOID:0050700 J:209752




Genotype
MGI:5702755
cn37
Allelic
Composition
Rock2tm1.1Itl/Rock2tm1.1Itl
Tg(Tagln-cre)1Her/0
Genetic
Background
involves: 129S/SvEv * C57BL/6 * C57BL/6J * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Rock2tm1.1Itl mutation (0 available); any Rock2 mutation (10 available)
Tg(Tagln-cre)1Her mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• time of lethality not specified




Genotype
MGI:5296817
cn38
Allelic
Composition
Atp1a2tm4.1Ling/Atp1a2tm4.1Ling
Tg(Tagln-cre)1Her/0
Genetic
Background
involves: Black Swiss * C57BL/6 * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Atp1a2tm4.1Ling mutation (0 available); any Atp1a2 mutation (2 available)
Tg(Tagln-cre)1Her mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
growth/size/body
• slight at 8 months of age
• mild at 8 months of age

cardiovascular system
N
• mice exhibit normal basal cardiac and vascular function
• slight at 8 months of age
• mild at 8 months of age
• mice fail to develop ACTH- or ouabain-induced hypertension unlike similarly treated wild-type mice
• however, basal systemic blood pressure is normal




Genotype
MGI:5910538
cn39
Allelic
Composition
Tg(CMV-cat,-ROCK2*)3-1Koba/0
Tg(Tagln-cre)1Her/0
Genetic
Background
involves: C57BL/6 * C57BL/6JJcl * DBA/2 * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(CMV-cat,-ROCK2*)3-1Koba mutation (0 available)
Tg(Tagln-cre)1Her mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
growth/size/body
• heart weight to body weight ratio is increased

mortality/aging
• 34.6% of mice die suddenly by 1 year of age
• some sudden death of several pups before weaning at 4 weeks of age is seen

cardiovascular system
• aortas are thinner
• widening of the gaps at the intercalated discs in the myocardium of 25 week old mice
• desmosomes are less electron-dense than in controls at 25 weeks of age and the gap width at the desmosomes is increased
• distribution of desmosomal proteins is altered
• right ventricle shows disorganized myocardial structure with poorly defined cell junctional areas
• massive fat accumulation in the right ventricle free wall adjacent to areas of fibrosis is seen in 19 week old mice
• heart weight to body weight ratio is increased
• ventricular weight to body weight ratio is increased
• thinning of interventricular septum of the hearts of E12.5 and E14.5 embryos
• massive fat accumulation in the right ventricle free wall adjacent to areas of fibrosis is seen in 19 week old mice
• thinning of ventricular walls of the hearts of E12.5 and E14.5 embryos
• myocardial fibrotic changes are first evident at P3 in both the right and left ventricles
• fibrosis is initially seen in epicardial and perivascular regions of the ventricles, progressing to adjacent myocardium with age and involving the entire thickness of the right ventricle free wall at 19 weeks of age
• hearts are markedly dilated as early as P3, with dilatation more prominent in the right ventricle and is progressive with age
• progressive ventricular dilatation and thinning from P3
• reduction in left ventricle ejection fraction and fractional shortening
• reduction in the number of proliferating cardiomyocytes in the ventricular walls and interventricular septum (both in compact layer and trabeculated layer) in E12.5 hearts
• however, apoptosis levels are normal
• echocardiography indicates dilated ventricular chambers, increased right ventricle and left ventricle dimensions, and reduced left ventricle ejection fraction and fractional shortening
• mice frequently exhibit spontaneous ventricular arrhythmias, evident as long frequent runs of ventricular extrasystoles characterized by widened QRS complexes with no apparent association with the P waves
• reduction in the number of proliferating cardiomyocytes in the ventricular walls and interventricular septum (both in compact layer and trabeculated layer) in adult hearts
• systolic blood pressure is lower
• mice develop arrhythmogenic right ventricular cardiomyopathy

cellular
• reduction in the number of proliferating cardiomyocytes in the ventricular walls and interventricular septum (both in compact layer and trabeculated layer) in E12.5 hearts
• however, apoptosis levels are normal

muscle
• widening of the gaps at the intercalated discs in the myocardium of 25 week old mice
• desmosomes are less electron-dense than in controls at 25 weeks of age and the gap width at the desmosomes is increased
• distribution of desmosomal proteins is altered
• right ventricle shows disorganized myocardial structure with poorly defined cell junctional areas
• massive fat accumulation in the right ventricle free wall adjacent to areas of fibrosis is seen in 19 week old mice
• reduction in left ventricle ejection fraction and fractional shortening
• reduction in the number of proliferating cardiomyocytes in the ventricular walls and interventricular septum (both in compact layer and trabeculated layer) in E12.5 hearts
• however, apoptosis levels are normal
• mice develop arrhythmogenic right ventricular cardiomyopathy

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
arrhythmogenic right ventricular cardiomyopathy DOID:0050431 OMIM:PS107970
J:242116




Genotype
MGI:5796351
cn40
Allelic
Composition
Commd9tm1c(KOMP)Wtsi/Commd9tm1c(KOMP)Wtsi
Tg(Tagln-cre)1Her/0
Genetic
Background
involves: C57BL/6 * C57BL/6N * SJL
Cell Lines EPD0136_6_D10
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Commd9tm1c(KOMP)Wtsi mutation (0 available); any Commd9 mutation (2 available)
Tg(Tagln-cre)1Her mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
N
• all mice are viable at birth




Genotype
MGI:4946108
cn41
Allelic
Composition
Agtr1atm1Uky/Agtr1atm1Uky
Tg(Tagln-cre)1Her/0
Genetic
Background
involves: C57BL/6 * C57BL/6N * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Agtr1atm1Uky mutation (1 available); any Agtr1a mutation (15 available)
Tg(Tagln-cre)1Her mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
N
• mice exhibit normal aortic structure




Genotype
MGI:5896388
cn42
Allelic
Composition
Sec24ctm1c(EUCOMM)Wtsi/Sec24ctm1c(EUCOMM)Wtsi
Tg(Tagln-cre)1Her/0
Genetic
Background
involves: C57BL/6 * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Sec24ctm1c(EUCOMM)Wtsi mutation (1 available); any Sec24c mutation (31 available)
Tg(Tagln-cre)1Her mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
normal phenotype
• viable and fertile




Genotype
MGI:4410314
cn43
Allelic
Composition
Efemp2tm1.1Tynk/Efemp2tm1.1Tynk
Tg(Tagln-cre)1Her/0
Genetic
Background
involves: C57BL/6 * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Efemp2tm1.1Tynk mutation (0 available); any Efemp2 mutation (18 available)
Tg(Tagln-cre)1Her mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• at P14, the elastic laminae is disrupted unlike in wild-type mice
• at 12 weeks, aorta are less distensible than in wild-type mice
• the elastic laminae is thicker and spongy with aberrant deposits in the interlaminar spaces unlike in wild-type mice
• at 12 weeks, aorta are less distensible and stiffer than in wild-type mice




Genotype
MGI:5707856
cn44
Allelic
Composition
Gata5tm1.1Nemr/Gata5tm1.1Nemr
Tg(Tagln-cre)1Her/0
Genetic
Background
involves: C57BL/6 * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gata5tm1.1Nemr mutation (0 available); any Gata5 mutation (10 available)
Tg(Tagln-cre)1Her mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
N
• mice exhibit normal blood pressure and vasodilatory response to acetylcholine





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last database update
04/06/2021
MGI 6.16
The Jackson Laboratory