Phenotypes associated with this allele
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Kmt2dtm1.1Kaig mutation
(1 available);
any
Kmt2d mutation
(167 available)
Tg(Tnnt2-cre)5Blh mutation
(1 available)
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mortality/aging
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• while present at E13.5, mice die by E14.5
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cardiovascular system
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N |
• mice exhibit normal septation of outflow tract into the aorta and pulmonary artery
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• at E11.5, ventricular myocytes exhibit prolonged waveforms of calcium transients compared with control cells
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cellular
muscle
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Nrastm1Tyj mutation
(1 available);
any
Nras mutation
(44 available)
Tg(Tnnt2-cre)5Blh mutation
(1 available)
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mortality/aging
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N |
• mice exhibit normal viability during embryonic development
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cardiovascular system
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N |
• mice do NOT exhibit any gross cardiac malformations during embryonic development
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tarbp2tm1.1Dzw mutation
(0 available);
any
Tarbp2 mutation
(22 available)
Tg(tetO-Mir208a)#Dzw mutation
(0 available)
Tg(Tnnt2-cre)5Blh mutation
(1 available)
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cardiovascular system
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N |
• transgenic expression of Mir208a prevents the onset of dilated cardiomyopathy in cardiac conditional knockouts of Tarbp2 and results in mice with normal heart morphology, histology, and function
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Prdm16tm1.1Brsp mutation
(1 available);
any
Prdm16 mutation
(72 available)
Tg(Tnnt2-cre)5Blh mutation
(1 available)
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cardiovascular system
mortality/aging
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• postnatal lethality before P7
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tarbp2tm1.1Dzw mutation
(0 available);
any
Tarbp2 mutation
(22 available)
Tg(Tnnt2-cre)5Blh mutation
(1 available)
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mortality/aging
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• Although homozygotes are born in normal mendelian ratio indicating no embryonic lethality, more than 80 percent of these cardiac conditional knockout homozygotes die by 4 months of age and none survive beyond 8 months of age
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cardiovascular system
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• although gross morphology of the heart is normal at 2 weeks of age, atrial dilation is found by 3 weeks of age and substantial dilation of both atrial and ventricular chambers is found by 1 month of age and is severe in the homozygotes that survive to 2 months of age, yet cardiomyocyte size appears normal
• neonatal adeno-associated virus-mediated delivery of Tarbp2 or transgenic expression of Mir208a suppresses chamber dilation, permits normal cardiac morphology and restores viability as does knockdown of Sox6, while Tnnt2-dirven viral-mediated overexpression of Sox6 recapitulates the phenotype of cardiac conditional null Tarbp2
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• by 3 weeks of age atrial dilation is found
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• by 1 month of age dilation of the ventricular chambers is also found
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• by 2 weeks of age the left ventricular fractional shortening is decreased and this drops precipitously resulting in severe systolic dysfunction by 1 month of age
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muscle
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• by 2 weeks of age the left ventricular fractional shortening is decreased and this drops precipitously resulting in severe systolic dysfunction by 1 month of age
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Fgf8tm1.3Mrt mutation
(1 available);
any
Fgf8 mutation
(18 available)
Fgf8tm1.4Mrt mutation
(0 available);
any
Fgf8 mutation
(18 available)
Tg(Tnnt2-cre)5Blh mutation
(1 available)
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cardiovascular system
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N |
• no outflow tract or right ventricle defects are observed at E10.5 and no outflow tract septation defects are seen in term fetuses
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
NipblGt(EUCE313f02)1.1Hmgu mutation
(0 available);
any
Nipbl mutation
(124 available)
Tg(Tnnt2-cre)5Blh mutation
(1 available)
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cardiovascular system
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N |
• heart size is similar to wild-type
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• in 30% of hearts at E17.5
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growth/size/body
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N |
• body size is similar to wild-type
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
NipblGt(EUCE313f02)Hmgu mutation
(0 available);
any
Nipbl mutation
(124 available)
Tg(Tnnt2-cre)5Blh mutation
(1 available)
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cardiovascular system
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• only a single heart showed an atrial septal defect, statistically indistinguishable from wild-type
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• correlates to body size
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growth/size/body
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mib1em1Jlp mutation
(0 available);
any
Mib1 mutation
(55 available)
Mib1tm2Kong mutation
(0 available);
any
Mib1 mutation
(55 available)
Tg(Tnnt2-cre)5Blh mutation
(1 available)
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cardiovascular system
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• noncompacted trabeculae in ventricular myocardium in adults
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• left ventricular compact layer 45% thinner in E16.5 embryos
• reduced compact-to-trabecular myocardium ratio in E16.5 embryos
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• various conotruncal defects in E16.5 embryos
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• myocardial fibrosis in septum and around coronary vessels in adults
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• reduced fractional shortening and ejection fraction in adults
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mortality/aging
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• only 5% of embryos develop to adulthood
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muscle
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• noncompacted trabeculae in ventricular myocardium in adults
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• left ventricular compact layer 45% thinner in E16.5 embryos
• reduced compact-to-trabecular myocardium ratio in E16.5 embryos
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• reduced fractional shortening and ejection fraction in adults
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pdlim5tm1Chen mutation
(0 available);
any
Pdlim5 mutation
(61 available)
Tg(Tnnt2-cre)5Blh mutation
(1 available)
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cardiovascular system
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• left ventricular chamber size is significantly enlarged
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• develop dilated cardiomyopathy starting at 3 months of age
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• left ventricular function significantly impaired as measured by fractional shortening
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muscle
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• develop dilated cardiomyopathy starting at 3 months of age
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• left ventricular function significantly impaired as measured by fractional shortening
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Hspb7tm1.1Chen mutation
(0 available);
any
Hspb7 mutation
(14 available)
Tg(Tnnt2-cre)5Blh mutation
(1 available)
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mortality/aging
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• all embryos died before E12.5 with an overall phenotype similar to that observed in Hspb7tm1.2Chen homozygotes; however no data are provided
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Lims1tm1.1Chen mutation
(0 available);
any
Lims1 mutation
(53 available)
Tg(Tnnt2-cre)5Blh mutation
(1 available)
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cardiovascular system
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N |
• mice exhibit normal heart morphology and physiology under normal conditions
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• following infarct induction
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• following infarct induction, mice exhibit reduced fractional shortening and increased infarct size and scar tissue with hemorrhage compared with similarly treated wild-type mice
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homeostasis/metabolism
muscle
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• following infarct induction
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cxadrtm1.1Mds mutation
(0 available);
any
Cxadr mutation
(15 available)
Cxadrtm1Mds mutation
(0 available);
any
Cxadr mutation
(15 available)
Tg(Tnnt2-cre)5Blh mutation
(1 available)
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mortality/aging
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• no live conditional null mice are found at 4 weeks after birth; embryos at E10.5; mice display identical phenotype to Cxadrtm1.1Mds homozygotes
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cardiovascular system
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• at E10.5, engorgement of the cardinal veins is apparent
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• hyperplasia of proximal heart tube is seen at E10.5
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• sinuatrial valves, located at the junction between sinus venosus and atrium in wild-type embryos, are absent
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cxadrtm1Mds mutation
(0 available);
any
Cxadr mutation
(15 available)
Tg(Tnnt2-cre)5Blh mutation
(1 available)
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mortality/aging
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• no live conditional null mice are found at 4 weeks after birth; embryos at E10.5; mice display identical phenotype to Cxadrtm1.1Mds homozygotes
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cardiovascular system
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• at E10.5, engorgement of the cardinal veins is apparent
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• hyperplasia of proximal heart tube is seen at E10.5
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• sinuatrial valves, located at the junction between sinus venosus and atrium in wild-type embryos, are absent
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Hand2tm1Cse mutation
(0 available);
any
Hand2 mutation
(12 available)
Tg(Tnnt2-cre)5Blh mutation
(1 available)
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mortality/aging
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• embryos survive at the expected Mendelian ratio until 9.5 dpc, with viablility dropping until 12.5 dpc after which no viable embryos are recovered
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cardiovascular system
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• at 12.5 dpc, surviving embryos show hypoplastic outflow tract
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• at 10.5 dpc severely affected embryos exhibit only a single clearly defined ventricle
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• at 12.5 dpc, surviving embryos show hypoplastic right ventricle
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mortality/aging
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• no mice are produced and only remnants of embryos are detected at E14.5
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Bmp4tm3.1Blh mutation
(0 available);
any
Bmp4 mutation
(21 available)
Tg(Tnnt2-cre)5Blh mutation
(1 available)
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mortality/aging
cardiovascular system
|
N |
• no outflow tract abnormalities are seen
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Fkbp1atm1.1Shou mutation
(0 available);
any
Fkbp1a mutation
(14 available)
Fkbp1atm1Zuk mutation
(0 available);
any
Fkbp1a mutation
(14 available)
Tg(Tnnt2-cre)5Blh mutation
(1 available)
|
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cardiovascular system
|
N |
• animals show normal ventricular chamber formation; normal trabeculation and compaction in ventricles are observed
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Bmp4tm1Blh mutation
(2 available);
any
Bmp4 mutation
(21 available)
Bmp4tm3.1Blh mutation
(0 available);
any
Bmp4 mutation
(21 available)
Tg(Tnnt2-cre)5Blh mutation
(1 available)
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mortality/aging
cardiovascular system
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• at E12.5 about a 20% decrease in proliferation is detected in the atrioventricular cushions but not in other areas of the heart
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• present in 80% of embryos at E15.5 - E16.5
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• single atrioventricular junction with a common valve
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Tnnt2-cre)5Blh mutation
(1 available)
Vegfatm2Gne mutation
(1 available);
any
Vegfa mutation
(37 available)
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mortality/aging
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• complete lethality is observed after E15.5
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growth/size/body
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• at E15.5, mutants are runted
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cardiovascular system
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• at E12.5, the peritruncal area and ventricular septum lack angiogenic sprouts or coronary plexuses that are observed in controls
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• by E14.5, mutants have only a few immature myocardial coronary arteries
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• dilated subepicardial veins at E14.5
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• at E15.5, mutants have necrotic or ruptured septa
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• at E15.5, mutants display cardiac hemorrhages
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muscle
cellular
|
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Lims1tm1.1Chen mutation
(0 available);
any
Lims1 mutation
(53 available)
Lims2tm1.1Chen mutation
(0 available);
any
Lims2 mutation
(20 available)
Tg(Tnnt2-cre)5Blh mutation
(1 available)
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mortality/aging
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• mice die within 4 weeks
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• mice die within 4 weeks
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cardiovascular system
|
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• mice show abnormalities in pectinate muscles in atrial appendages
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• cultured attached cardiomyocytes are smaller than similarly treated wild-type cells
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• significantly thinner compact myocardium at P1
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• at P1, myocardium exhibit widened gaps of intercalated disks with disarrayed sarcomeres and distorted Z lines compared to in wild-type mice
• at P10, membranes of the intercalated disk are highly convoluted and gaps are widened unlike in wild-type mice
• the myocardium exhibits disruption of cell-cell adhesion compared to in wild-type mice
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• hearts are abnormally shaped with a bulge at the base of the right ventricle at P20
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• hearts are rounder at P1
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• mice exhibit disorganized trabeculae that fail to fuse and incorporate into compact myocardium unlike in wild-type mice
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• at P20, the ventricular wall exhibits irregular thickness with a thickened right ventricular free wall and thin left ventricular free wall and septum compared to in wild-type mice
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• at P20, hearts show fibrosis, esp. in the ventricular septum and within thinned regions of ventricular myocardium
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• few cardiomyocytes attack to a plate coated in fibronectin, laminin, and collagen compared with wild-type cells
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• progressive, resulting in death
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homeostasis/metabolism
liver/biliary system
respiratory system
growth/size/body
muscle
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• mice show abnormalities in pectinate muscles in atrial appendages
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• cultured attached cardiomyocytes are smaller than similarly treated wild-type cells
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• significantly thinner compact myocardium at P1
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• at P1, myocardium exhibit widened gaps of intercalated disks with disarrayed sarcomeres and distorted Z lines compared to in wild-type mice
• at P10, membranes of the intercalated disk are highly convoluted and gaps are widened unlike in wild-type mice
• the myocardium exhibits disruption of cell-cell adhesion compared to in wild-type mice
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Smotm2.1Amc mutation
(0 available);
any
Smo mutation
(39 available)
Smotm2Amc mutation
(1 available);
any
Smo mutation
(39 available)
Tg(Tnnt2-cre)5Blh mutation
(1 available)
|
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cardiovascular system
|
N |
• mice exhibit normal outflow tract development
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gt(ROSA)26Sortm1(CAG-Mlip)Dzw mutation
(0 available);
any
Gt(ROSA)26Sor mutation
(942 available)
Tg(Tnnt2-cre)5Blh mutation
(1 available)
|
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cardiovascular system
|
N |
• no cardiac phenotype under baseline conditions
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• at 4 weeks after transverse aortic constriction (TAC) cardiac hypertrophy is suppressed and cardiac function is preserved compared to similarly treated wild-type controls
• cardiac function is preserved and no signs of left ventricular dilation are seen after 10 weeks of TAC unlike in controls
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homeostasis/metabolism
|
|
| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Nexntm1Chen mutation
(0 available);
any
Nexn mutation
(28 available)
Tg(Tnnt2-cre)5Blh mutation
(1 available)
|
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mortality/aging
|
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• all mice die before P12
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cardiovascular system
|
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• mice develop progressive dilated cardiomyopathy
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muscle
|
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• mice develop progressive dilated cardiomyopathy
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Bicraem3Hzhg mutation
(0 available);
any
Bicra mutation
(55 available)
Tg(Tnnt2-cre)5Blh mutation
(1 available)
|
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mortality/aging
|
N |
• mice exhibit normal embryonic survival
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cardiovascular system
|
N |
• mice exhibit normal cardiac phenotype
|
|
|
| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cenpftm1Dbdr mutation
(0 available);
any
Cenpf mutation
(109 available)
Tg(Tnnt2-cre)5Blh mutation
(1 available)
|
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cardiovascular system
|
N |
• development of heart structure is grossly normal
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• modest reduction in coronary vasculature
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• costamere structure is impaired
• decreased number of myocytes in adults
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• intercalated disc number is reduced 3.5 fold in mature ventricles
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• trabeculae are thinner and blunted
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• weight at 4 days of age is 8.2mg compared to 10.5mg for controls
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• internal dimension somewhat increased
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• modest reduction in epicardial thickness
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• cardiac fibrosis in adults
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• decreasing ventricular function with age
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• mice develop progressive dilated cardiomyopathy
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• three fold decrease in prenatal myocardiocyte proliferation
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• P-P intervals greater than 200 ms
• slowing sinus rhythms
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• lower myocyte mitotic rates on days 1-4 after birth
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growth/size/body
|
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• normal to slightly smaller body size
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mortality/aging
|
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• 20% mortality in the 12 to 21 month age period
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muscle
|
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• costamere structure is impaired
• decreased number of myocytes in adults
|
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• intercalated disc number is reduced 3.5 fold in mature ventricles
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• trabeculae are thinner and blunted
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• mice develop progressive dilated cardiomyopathy
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• three fold decrease in prenatal myocardiocyte proliferation
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cellular
|
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• three fold decrease in prenatal myocardiocyte proliferation
|
Analysis Tools