All Phenotypes Tg(Tnnt2-cre)5Blh MGI Mouse

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Phenotypes associated with this allele

Allele Symbol
Allele Name
Allele ID

Tg(Tnnt2-cre)5Blh
transgene insertion 5, Brigid L Hogan
MGI:2679081

Summary

26 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
cn1	Kmt2d^tm1.1Kaig/Kmt2d^tm1.1Kaig Tg(Tnnt2-cre)5Blh/0	B6.Cg-Kmt2d^tm1.1Kaig Tg(Tnnt2-cre)5Blh	MGI:5780098
cn2	Nras^tm1Tyj/Nras⁺ Tg(Tnnt2-cre)5Blh/0	B6.Cg-Nras^tm1Tyj Tg(Tnnt2-cre)5Blh	MGI:7544844
cn3	Tarbp2^tm1.1Dzw/Tarbp2^tm1.1Dzw Tg(tetO-Mir208a)#Dzw/? Tg(Tnnt2-cre)5Blh/?	involves: 129 * C3H * C57BL/6 * DBA/2J	MGI:5645730
cn4	Prdm16^tm1.1Brsp/Prdm16^tm1.1Brsp Tg(Tnnt2-cre)5Blh/0	involves: 129 * C57BL/6 * C57BL/6J * DBA/2	MGI:7314697
cn5	Tarbp2^tm1.1Dzw/Tarbp2^tm1.1Dzw Tg(Tnnt2-cre)5Blh/0	involves: 129 * C57BL/6 * DBA/2J	MGI:5645529
cn6	Fgf8^tm1.3Mrt/Fgf8^tm1.4Mrt Tg(Tnnt2-cre)5Blh/0	involves: 129P2/OlaHsd * 129S7/SvEvBrd * C57BL/6 * DBA/2	MGI:3818074
cn7	Nipbl^{Gt(EUCE313f02)1.1Hmgu}/Nipbl⁺ Tg(Tnnt2-cre)5Blh/0	involves: 129P2/OlaHsd * C57BL/6 * CD-1 * DBA/2	MGI:7492222
cn8	Nipbl^{Gt(EUCE313f02)Hmgu}/Nipbl⁺ Tg(Tnnt2-cre)5Blh/0	involves: 129P2/OlaHsd * C57BL/6 * CD-1 * DBA/2	MGI:7492241
cn9	Mib1^em1Jlp/Mib1^tm2Kong Tg(Tnnt2-cre)5Blh/0	involves: 129P2/OlaHsd * C57BL/6 * DBA/2	MGI:7544909
cn10	Pdlim5^tm1Chen/Pdlim5^tm1Chen Tg(Tnnt2-cre)5Blh/?	involves: 129S1/Sv * 129X1/SvJ * Black Swiss * C57BL/6 * DBA/2	MGI:5284901
cn11	Cxadr^tm1Mds/Cxadr^tm1.1Mds Tg(Tnnt2-cre)5Blh/0	involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * DBA/2	MGI:3711513
cn12	Cxadr^tm1Mds/Cxadr^tm1Mds Tg(Tnnt2-cre)5Blh/0	involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * DBA/2	MGI:3711514
cn13	Lims1^tm1.1Chen/Lims1^tm1.1Chen Tg(Tnnt2-cre)5Blh/0	involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * DBA/2	MGI:4867271
cn14	Hspb7^tm1.1Chen/Hspb7^tm1.1Chen Tg(Tnnt2-cre)5Blh/0	involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * DBA/2	MGI:6159010
cn15	Hand2^tm1Cse/Hand2⁺ Tg(Tnnt2-cre)5Blh/0	involves: 129S1/Sv * C57BL/6J * DBA/2	MGI:4417974
cn16	Myo18a^{tm1c(KOMP)Wtsi}/Myo18a^{tm1c(KOMP)Wtsi} Gt(ROSA)26Sor^tm1(EYFP)Cos/Gt(ROSA)26Sor⁺ Tg(Tnnt2-cre)5Blh/0	involves: 129S4/SvJaeSor * 129X1/SvJ * C57BL/6 * C57BL/6N * DBA/2	MGI:6360587
cn17	Bmp4^tm3.1Blh/Bmp4^tm3.1Blh Tg(Tnnt2-cre)5Blh/0	involves: 129S6/SvEvTac * C57BL/6 * DBA/2 * ICR	MGI:2679083
cn18	Fkbp1a^tm1.1Shou/Fkbp1a^tm1Zuk Tg(Tnnt2-cre)5Blh/0	involves: 129S7/SvEvBrd * C57BL/6 * DBA/2	MGI:5490248
cn19	Bmp4^tm1Blh/Bmp4^tm3.1Blh Tg(Tnnt2-cre)5Blh/0	involves: 129S/Sv * Black Swiss * C57BL/6 * DBA/2 * ICR	MGI:2679084
cn20	Vegfa^tm2Gne/Vegfa^tm2Gne Tg(Tnnt2-cre)5Blh/0	involves: 129/Sv * C57BL/6 * DBA	MGI:5471120
cn21	Lims1^tm1.1Chen/Lims1^tm1.1Chen Lims2^tm1.1Chen/Lims2^tm1.1Chen Tg(Tnnt2-cre)5Blh/0	involves: 129/Sv * C57BL/6 * DBA/2	MGI:4867270
cn22	Smo^tm2Amc/Smo^tm2.1Amc Tg(Tnnt2-cre)5Blh/0	involves: 129X1/SvJ * C57BL/6 * DBA/2	MGI:4843922
cn23	Gt(ROSA)26Sor^{tm1(CAG-Mlip)Dzw}/Gt(ROSA)26Sor⁺ Tg(Tnnt2-cre)5Blh/0	involves: C57BL/6 * DBA/2	MGI:5907356
cn24	Nexn^tm1Chen/Nexn^tm1Chen Tg(Tnnt2-cre)5Blh/0	involves: C57BL/6 * DBA/2	MGI:6514900
cn25	Bicra^em3Hzhg/Bicra^em3Hzhg Tg(Tnnt2-cre)5Blh/0	involves: C57BL/6 * DBA/2	MGI:7543773
cn26	Cenpf^tm1Dbdr/Cenpf^tm1Dbdr Tg(Tnnt2-cre)5Blh/0	involves: C57BL/6 * DBA/2 * ICR	MGI:5467575

Allelic Composition	Kmt2d^tm1.1Kaig/Kmt2d^tm1.1Kaig Tg(Tnnt2-cre)5Blh/0
Genetic Background	B6.Cg-Kmt2d^tm1.1Kaig Tg(Tnnt2-cre)5Blh

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Kmt2d^tm1.1Kaig mutation (1 available); any Kmt2d mutation (169 available)
Tg(Tnnt2-cre)5Blh mutation (1 available)

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

mortality/aging

embryonic lethality during organogenesis, complete penetrance ( J:229890 )

• while present at E13.5, mice die by E14.5

cardiovascular system

cardiovascular system phenotype ( J:229890 )

N	• mice exhibit normal septation of outflow tract into the aorta and pulmonary artery

thin myocardium compact layer ( J:229890 )

• at E13.5

abnormal interventricular septum morphology ( J:229890 )

• disorganized

abnormal fetal cardiomyocyte physiology ( J:229890 )

• at E11.5, ventricular myocytes exhibit prolonged waveforms of calcium transients compared with control cells

decreased fetal cardiomyocyte proliferation ( J:229890 )

cellular

decreased fetal cardiomyocyte proliferation ( J:229890 )

muscle

thin myocardium compact layer ( J:229890 )

• at E13.5

decreased fetal cardiomyocyte proliferation ( J:229890 )

Allelic Composition	Nras^tm1Tyj/Nras⁺ Tg(Tnnt2-cre)5Blh/0
Genetic Background	B6.Cg-Nras^tm1Tyj Tg(Tnnt2-cre)5Blh

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Nras^tm1Tyj mutation (1 available); any Nras mutation (44 available)
Tg(Tnnt2-cre)5Blh mutation (1 available)

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

mortality/aging

mortality/aging ( J:305401 )

N	• mice exhibit normal viability during embryonic development

cardiovascular system

cardiovascular system phenotype ( J:305401 )

N	• mice do NOT exhibit any gross cardiac malformations during embryonic development

Allelic Composition	Tarbp2^tm1.1Dzw/Tarbp2^tm1.1Dzw Tg(tetO-Mir208a)#Dzw/? Tg(Tnnt2-cre)5Blh/?
Genetic Background	involves: 129 * C3H * C57BL/6 * DBA/2J

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tarbp2^tm1.1Dzw mutation (0 available); any Tarbp2 mutation (22 available)
Tg(tetO-Mir208a)#Dzw mutation (0 available)
Tg(Tnnt2-cre)5Blh mutation (1 available)

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

cardiovascular system

cardiovascular system phenotype ( J:222613 )

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

Allelic Composition	Prdm16^tm1.1Brsp/Prdm16^tm1.1Brsp Tg(Tnnt2-cre)5Blh/0
Genetic Background	involves: 129 * C57BL/6 * C57BL/6J * DBA/2

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Prdm16^tm1.1Brsp mutation (1 available); any Prdm16 mutation (73 available)
Tg(Tnnt2-cre)5Blh mutation (1 available)

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

cardiovascular system

dilated heart left ventricle ( J:326525 )

mortality/aging

postnatal lethality, complete penetrance ( J:326525 )

• postnatal lethality before P7

Allelic Composition	Tarbp2^tm1.1Dzw/Tarbp2^tm1.1Dzw Tg(Tnnt2-cre)5Blh/0
Genetic Background	involves: 129 * C57BL/6 * DBA/2J

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tarbp2^tm1.1Dzw mutation (0 available); any Tarbp2 mutation (22 available)
Tg(Tnnt2-cre)5Blh mutation (1 available)

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

mortality/aging

premature death ( J:222613 )

• 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

cardiovascular system

cardiac fibrosis ( J:222613 )

dilated heart ( J:222613 )

• 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

dilated heart atrium ( J:222613 )

• by 3 weeks of age atrial dilation is found

dilated heart ventricle ( J:222613 )

• by 1 month of age dilation of the ventricular chambers is also found

dilated cardiomyopathy ( J:222613 )

decreased heart left ventricle muscle contractility ( J:222613 )

• 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

muscle

dilated cardiomyopathy ( J:222613 )

decreased heart left ventricle muscle contractility ( J:222613 )

• 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

Allelic Composition	Fgf8^tm1.3Mrt/Fgf8^tm1.4Mrt Tg(Tnnt2-cre)5Blh/0
Genetic Background	involves: 129P2/OlaHsd * 129S7/SvEvBrd * C57BL/6 * DBA/2

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Fgf8^tm1.3Mrt mutation (1 available); any Fgf8 mutation (21 available)
Fgf8^tm1.4Mrt mutation (0 available); any Fgf8 mutation (21 available)
Tg(Tnnt2-cre)5Blh mutation (1 available)

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

cardiovascular system

cardiovascular system phenotype ( J:109474 )

N	• no outflow tract or right ventricle defects are observed at E10.5 and no outflow tract septation defects are seen in term fetuses

Allelic Composition	Nipbl^{Gt(EUCE313f02)1.1Hmgu}/Nipbl⁺ Tg(Tnnt2-cre)5Blh/0
Genetic Background	involves: 129P2/OlaHsd * C57BL/6 * CD-1 * DBA/2

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Nipbl^{Gt(EUCE313f02)1.1Hmgu} mutation (0 available); any Nipbl mutation (124 available)
Tg(Tnnt2-cre)5Blh mutation (1 available)

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

cardiovascular system

cardiovascular system phenotype ( J:236978 )

N	• heart size is similar to wild-type

atrial septal defect ( J:236978 )

• in 30% of hearts at E17.5

growth/size/body

growth/size/body region phenotype ( J:236978 )

N	• body size is similar to wild-type

Allelic Composition	Nipbl^{Gt(EUCE313f02)Hmgu}/Nipbl⁺ Tg(Tnnt2-cre)5Blh/0
Genetic Background	involves: 129P2/OlaHsd * C57BL/6 * CD-1 * DBA/2

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Nipbl^{Gt(EUCE313f02)Hmgu} mutation (0 available); any Nipbl mutation (124 available)
Tg(Tnnt2-cre)5Blh mutation (1 available)

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

cardiovascular system

cardiovascular system phenotype ( J:236978 )

• only a single heart showed an atrial septal defect, statistically indistinguishable from wild-type

small heart ( J:236978 )

• correlates to body size

growth/size/body

decreased body size ( J:236978 )

Allelic Composition	Mib1^em1Jlp/Mib1^tm2Kong Tg(Tnnt2-cre)5Blh/0
Genetic Background	involves: 129P2/OlaHsd * C57BL/6 * DBA/2

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mib1^em1Jlp mutation (0 available); any Mib1 mutation (55 available)
Mib1^tm2Kong mutation (0 available); any Mib1 mutation (55 available)
Tg(Tnnt2-cre)5Blh mutation (1 available)

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

cardiovascular system

abnormal trabecula carnea morphology ( J:338889 )

• noncompacted trabeculae in ventricular myocardium in adults

thin left ventricle myocardium compact layer ( J:338889 )

• left ventricular compact layer 45% thinner in E16.5 embryos

• reduced compact-to-trabecular myocardium ratio in E16.5 embryos

abnormal cardiac outflow tract development ( J:338889 )

• various conotruncal defects in E16.5 embryos

abnormal aortic valve morphology ( J:338889 )

• dysplasia in adults

bicuspid aortic valve ( J:338889 )

• in E16.5 embryos

dilated heart ventricle ( J:338889 )

• in adults

cardiac fibrosis ( J:338889 )

• myocardial fibrosis in septum and around coronary vessels in adults

decreased cardiac muscle contractility ( J:338889 )

• reduced fractional shortening and ejection fraction in adults

aortic valve regurgitation ( J:338889 )

• in adults

mortality/aging

lethality during fetal growth through weaning, incomplete penetrance ( J:338889 )

• only 5% of embryos develop to adulthood

muscle

abnormal trabecula carnea morphology ( J:338889 )

• noncompacted trabeculae in ventricular myocardium in adults

thin left ventricle myocardium compact layer ( J:338889 )

• left ventricular compact layer 45% thinner in E16.5 embryos

• reduced compact-to-trabecular myocardium ratio in E16.5 embryos

decreased cardiac muscle contractility ( J:338889 )

• reduced fractional shortening and ejection fraction in adults

Allelic Composition	Pdlim5^tm1Chen/Pdlim5^tm1Chen Tg(Tnnt2-cre)5Blh/?
Genetic Background	involves: 129S1/Sv * 129X1/SvJ * Black Swiss * C57BL/6 * DBA/2

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pdlim5^tm1Chen mutation (0 available); any Pdlim5 mutation (62 available)
Tg(Tnnt2-cre)5Blh mutation (1 available)

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

cardiovascular system

dilated heart left ventricle ( J:175042 )

• left ventricular chamber size is significantly enlarged

dilated cardiomyopathy ( J:175042 )

• develop dilated cardiomyopathy starting at 3 months of age

decreased heart left ventricle muscle contractility ( J:175042 )

• left ventricular function significantly impaired as measured by fractional shortening

muscle

dilated cardiomyopathy ( J:175042 )

• develop dilated cardiomyopathy starting at 3 months of age

decreased heart left ventricle muscle contractility ( J:175042 )

• left ventricular function significantly impaired as measured by fractional shortening

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
dilated cardiomyopathy		DOID:12930	OMIM:PS115200	J:175042

Allelic Composition	Cxadr^tm1Mds/Cxadr^tm1.1Mds Tg(Tnnt2-cre)5Blh/0
Genetic Background	involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * DBA/2

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cxadr^tm1.1Mds mutation (0 available); any Cxadr mutation (15 available)
Cxadr^tm1Mds mutation (0 available); any Cxadr mutation (15 available)
Tg(Tnnt2-cre)5Blh mutation (1 available)

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

mortality/aging

embryonic lethality during organogenesis, complete penetrance ( J:121400 )

• no live conditional null mice are found at 4 weeks after birth; embryos at E10.5; mice display identical phenotype to Cxadr^tm1.1Mds homozygotes

cardiovascular system

abnormal cardinal vein morphology ( J:121400 )

• at E10.5, engorgement of the cardinal veins is apparent

abnormal heart tube morphology ( J:121400 )

• hyperplasia of proximal heart tube is seen at E10.5

absent sinoatrial valve ( J:121400 )

• sinuatrial valves, located at the junction between sinus venosus and atrium in wild-type embryos, are absent

Allelic Composition	Cxadr^tm1Mds/Cxadr^tm1Mds Tg(Tnnt2-cre)5Blh/0
Genetic Background	involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * DBA/2

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cxadr^tm1Mds mutation (0 available); any Cxadr mutation (15 available)
Tg(Tnnt2-cre)5Blh mutation (1 available)

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

mortality/aging

embryonic lethality during organogenesis, complete penetrance ( J:121400 )

• no live conditional null mice are found at 4 weeks after birth; embryos at E10.5; mice display identical phenotype to Cxadr^tm1.1Mds homozygotes

cardiovascular system

abnormal cardinal vein morphology ( J:121400 )

• at E10.5, engorgement of the cardinal veins is apparent

abnormal heart tube morphology ( J:121400 )

• hyperplasia of proximal heart tube is seen at E10.5

absent sinoatrial valve ( J:121400 )

• sinuatrial valves, located at the junction between sinus venosus and atrium in wild-type embryos, are absent

Allelic Composition	Lims1^tm1.1Chen/Lims1^tm1.1Chen Tg(Tnnt2-cre)5Blh/0
Genetic Background	involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * DBA/2

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Lims1^tm1.1Chen mutation (0 available); any Lims1 mutation (53 available)
Tg(Tnnt2-cre)5Blh mutation (1 available)

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

cardiovascular system

cardiovascular system phenotype ( J:167114 )

N	• mice exhibit normal heart morphology and physiology under normal conditions

decreased cardiac muscle contractility ( J:167114 )

• following infarct induction

abnormal response to cardiac infarction ( J:167114 )

• following infarct induction, mice exhibit reduced fractional shortening and increased infarct size and scar tissue with hemorrhage compared with similarly treated wild-type mice

increased myocardial infarct size ( J:167114 )

homeostasis/metabolism

abnormal response to cardiac infarction ( J:167114 )

• following infarct induction, mice exhibit reduced fractional shortening and increased infarct size and scar tissue with hemorrhage compared with similarly treated wild-type mice

increased myocardial infarct size ( J:167114 )

muscle

decreased cardiac muscle contractility ( J:167114 )

• following infarct induction

Allelic Composition	Hspb7^tm1.1Chen/Hspb7^tm1.1Chen Tg(Tnnt2-cre)5Blh/0
Genetic Background	involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * DBA/2

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Hspb7^tm1.1Chen mutation (0 available); any Hspb7 mutation (14 available)
Tg(Tnnt2-cre)5Blh mutation (1 available)

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

mortality/aging

embryonic lethality during organogenesis, complete penetrance ( J:256653 )

• all embryos died before E12.5 with an overall phenotype similar to that observed in Hspb7^tm1.2Chen homozygotes; however no data are provided

Allelic Composition	Hand2^tm1Cse/Hand2⁺ Tg(Tnnt2-cre)5Blh/0
Genetic Background	involves: 129S1/Sv * C57BL/6J * DBA/2

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Hand2^tm1Cse mutation (0 available); any Hand2 mutation (12 available)
Tg(Tnnt2-cre)5Blh mutation (1 available)

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

mortality/aging

embryonic lethality during organogenesis, complete penetrance ( J:155265 )

• 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

cardiovascular system

abnormal cardiac outflow tract development ( J:155265 )

• at 12.5 dpc, surviving embryos show hypoplastic outflow tract

abnormal heart ventricle morphology ( J:155265 )

• at 10.5 dpc severely affected embryos exhibit only a single clearly defined ventricle

decreased heart right ventricle size ( J:155265 )

• at 12.5 dpc, surviving embryos show hypoplastic right ventricle

Allelic Composition	Myo18a^{tm1c(KOMP)Wtsi}/Myo18a^{tm1c(KOMP)Wtsi} Gt(ROSA)26Sor^tm1(EYFP)Cos/Gt(ROSA)26Sor⁺ Tg(Tnnt2-cre)5Blh/0
Genetic Background	involves: 129S4/SvJaeSor * 129X1/SvJ * C57BL/6 * C57BL/6N * DBA/2

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gt(ROSA)26Sor^tm1(EYFP)Cos mutation (11 available); any Gt(ROSA)26Sor mutation (993 available)
Myo18a^{tm1c(KOMP)Wtsi} mutation (1 available); any Myo18a mutation (124 available)
Tg(Tnnt2-cre)5Blh mutation (1 available)

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

mortality/aging

lethality throughout fetal growth and development, complete penetrance ( J:277320 )

• no mice are produced and only remnants of embryos are detected at E14.5

Allelic Composition	Bmp4^tm3.1Blh/Bmp4^tm3.1Blh Tg(Tnnt2-cre)5Blh/0
Genetic Background	involves: 129S6/SvEvTac * C57BL/6 * DBA/2 * ICR

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Bmp4^tm3.1Blh mutation (0 available); any Bmp4 mutation (23 available)
Tg(Tnnt2-cre)5Blh mutation (1 available)

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

mortality/aging

perinatal lethality ( J:86001 )

cardiovascular system

cardiovascular system phenotype ( J:86001 )

N	• no outflow tract abnormalities are seen

atrial septal defect ( J:86001 )

• atrial septal defect similar to that in mice heterozygous for Bmp4^tm1Blh and Bmp4^tm3.1Blh

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
atrioventricular septal defect		DOID:0050651	OMIM:606215 OMIM:614430 OMIM:614474	J:86001

Allelic Composition	Fkbp1a^tm1.1Shou/Fkbp1a^tm1Zuk Tg(Tnnt2-cre)5Blh/0
Genetic Background	involves: 129S7/SvEvBrd * C57BL/6 * DBA/2

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Fkbp1a^tm1.1Shou mutation (0 available); any Fkbp1a mutation (14 available)
Fkbp1a^tm1Zuk mutation (0 available); any Fkbp1a mutation (14 available)
Tg(Tnnt2-cre)5Blh mutation (1 available)

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

cardiovascular system

cardiovascular system phenotype ( J:195489 )

N	• animals show normal ventricular chamber formation; normal trabeculation and compaction in ventricles are observed

Allelic Composition	Bmp4^tm1Blh/Bmp4^tm3.1Blh Tg(Tnnt2-cre)5Blh/0
Genetic Background	involves: 129S/Sv * Black Swiss * C57BL/6 * DBA/2 * ICR

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Bmp4^tm1Blh mutation (2 available); any Bmp4 mutation (23 available)
Bmp4^tm3.1Blh mutation (0 available); any Bmp4 mutation (23 available)
Tg(Tnnt2-cre)5Blh mutation (1 available)

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

mortality/aging

perinatal lethality, complete penetrance ( J:86001 )

• no neonates are found

cardiovascular system

abnormal atrioventricular cushion morphology ( J:86001 )

• at E12.5 about a 20% decrease in proliferation is detected in the atrioventricular cushions but not in other areas of the heart

double outlet right ventricle ( J:86001 )

• present in 80% of embryos at E15.5 - E16.5

common atrioventricular valve ( J:86001 )

• single atrioventricular junction with a common valve

complete atrioventricular septal defect ( J:86001 )

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
atrioventricular septal defect		DOID:0050651	OMIM:606215 OMIM:614430 OMIM:614474	J:86001

Allelic Composition	Vegfa^tm2Gne/Vegfa^tm2Gne Tg(Tnnt2-cre)5Blh/0
Genetic Background	involves: 129/Sv * C57BL/6 * DBA

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Tnnt2-cre)5Blh mutation (1 available)
Vegfa^tm2Gne mutation (2 available); any Vegfa mutation (38 available)

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

mortality/aging

lethality throughout fetal growth and development, complete penetrance ( J:193198 )

• complete lethality is observed after E15.5

growth/size/body

decreased body size ( J:193198 )

• at E15.5, mutants are runted

cardiovascular system

cardiovascular system phenotype ( J:193198 )

abnormal angiogenesis ( J:193198 )

• at E12.5, the peritruncal area and ventricular septum lack angiogenic sprouts or coronary plexuses that are observed in controls

myocardium necrosis ( J:193198 )

abnormal coronary vessel morphology ( J:193198 )

abnormal coronary artery morphology ( J:193198 )

• by E14.5, mutants have only a few immature myocardial coronary arteries

abnormal coronary vein morphology ( J:193198 )

• dilated subepicardial veins at E14.5

abnormal heart septum morphology ( J:193198 )

• at E15.5, mutants have necrotic or ruptured septa

thin ventricular wall ( J:193198 )

heart hemorrhage ( J:193198 )

• at E15.5, mutants display cardiac hemorrhages

muscle

myocardium necrosis ( J:193198 )

Allelic Composition	Lims1^tm1.1Chen/Lims1^tm1.1Chen Lims2^tm1.1Chen/Lims2^tm1.1Chen Tg(Tnnt2-cre)5Blh/0
Genetic Background	involves: 129/Sv * C57BL/6 * DBA/2

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Lims1^tm1.1Chen mutation (0 available); any Lims1 mutation (53 available)
Lims2^tm1.1Chen mutation (0 available); any Lims2 mutation (20 available)
Tg(Tnnt2-cre)5Blh mutation (1 available)

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

mortality/aging

premature death ( J:167114 )

• mice die within 4 weeks

postnatal lethality, incomplete penetrance ( J:167114 )

• mice die within 4 weeks

cardiovascular system

liver vascular congestion ( J:167114 )

abnormal pectinate muscle morphology ( J:167114 )

• mice show abnormalities in pectinate muscles in atrial appendages

decreased myocardial fiber size ( J:167114 )

• cultured attached cardiomyocytes are smaller than similarly treated wild-type cells

thin myocardium compact layer ( J:167114 )

• significantly thinner compact myocardium at P1

abnormal ventricle myocardium morphology ( J:167114 )

• 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

dilated heart atrium ( J:167114 )

• at P20

abnormal heart shape ( J:167114 )

• hearts are abnormally shaped with a bulge at the base of the right ventricle at P20

globular heart ( J:167114 )

• hearts are rounder at P1

abnormal heart left ventricle morphology ( J:167114 )

• mice exhibit disorganized trabeculae that fail to fuse and incorporate into compact myocardium unlike in wild-type mice

dilated heart left ventricle ( J:167114 )

• at P1 and P20

abnormal heart ventricle wall thickness ( J:167114 )

• 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

cardiac fibrosis ( J:167114 )

• at P20, hearts show fibrosis, esp. in the ventricular septum and within thinned regions of ventricular myocardium

abnormal myocardial fiber physiology ( J:167114 )

• few cardiomyocytes attack to a plate coated in fibronectin, laminin, and collagen compared with wild-type cells

congestive heart failure ( J:167114 )

• progressive, resulting in death

homeostasis/metabolism

abnormal atrial thrombosis ( J:167114 )

• at P20, a large intracardiac thrombus is frequently detected in the left atria

edema ( J:167114 )

• extensive in the lungs and liver

pulmonary edema ( J:167114 )

ascites ( J:167114 )

liver/biliary system

liver vascular congestion ( J:167114 )

enlarged liver ( J:167114 )

respiratory system

increased lung weight ( J:167114 )

pulmonary edema ( J:167114 )

growth/size/body

postnatal growth retardation ( J:167114 )

• gradual

increased lung weight ( J:167114 )

enlarged liver ( J:167114 )

muscle

abnormal pectinate muscle morphology ( J:167114 )

• mice show abnormalities in pectinate muscles in atrial appendages

decreased myocardial fiber size ( J:167114 )

• cultured attached cardiomyocytes are smaller than similarly treated wild-type cells

thin myocardium compact layer ( J:167114 )

• significantly thinner compact myocardium at P1

abnormal ventricle myocardium morphology ( J:167114 )

• 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

Allelic Composition	Smo^tm2Amc/Smo^tm2.1Amc Tg(Tnnt2-cre)5Blh/0
Genetic Background	involves: 129X1/SvJ * C57BL/6 * DBA/2

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Smo^tm2.1Amc mutation (0 available); any Smo mutation (39 available)
Smo^tm2Amc mutation (1 available); any Smo mutation (39 available)
Tg(Tnnt2-cre)5Blh mutation (1 available)

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

cardiovascular system

cardiovascular system phenotype ( J:135134 )

N	• mice exhibit normal outflow tract development

Allelic Composition	Gt(ROSA)26Sor^{tm1(CAG-Mlip)Dzw}/Gt(ROSA)26Sor⁺ Tg(Tnnt2-cre)5Blh/0
Genetic Background	involves: C57BL/6 * DBA/2

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gt(ROSA)26Sor^{tm1(CAG-Mlip)Dzw} mutation (0 available); any Gt(ROSA)26Sor mutation (993 available)
Tg(Tnnt2-cre)5Blh mutation (1 available)

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

cardiovascular system

cardiovascular system phenotype ( J:227485 )

N	• no cardiac phenotype under baseline conditions

decreased response of heart to induced stress ( J:227485 )

• 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

homeostasis/metabolism

decreased response of heart to induced stress ( J:227485 )

• 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

Allelic Composition	Nexn^tm1Chen/Nexn^tm1Chen Tg(Tnnt2-cre)5Blh/0
Genetic Background	involves: C57BL/6 * DBA/2

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Nexn^tm1Chen mutation (0 available); any Nexn mutation (28 available)
Tg(Tnnt2-cre)5Blh mutation (1 available)

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

mortality/aging

postnatal lethality, complete penetrance ( J:290931 )

• all mice die before P12

cardiovascular system

dilated cardiomyopathy ( J:290931 )

• mice develop progressive dilated cardiomyopathy

muscle

dilated cardiomyopathy ( J:290931 )

• mice develop progressive dilated cardiomyopathy

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
dilated cardiomyopathy 1CC		DOID:0110424	OMIM:613122	J:290931

Allelic Composition	Bicra^em3Hzhg/Bicra^em3Hzhg Tg(Tnnt2-cre)5Blh/0
Genetic Background	involves: C57BL/6 * DBA/2

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Bicra^em3Hzhg mutation (0 available); any Bicra mutation (55 available)
Tg(Tnnt2-cre)5Blh mutation (1 available)

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

mortality/aging

mortality/aging ( J:335098 )

N	• mice exhibit normal embryonic survival

cardiovascular system

cardiovascular system phenotype ( J:335098 )

N	• mice exhibit normal cardiac phenotype

Allelic Composition	Cenpf^tm1Dbdr/Cenpf^tm1Dbdr Tg(Tnnt2-cre)5Blh/0
Genetic Background	involves: C57BL/6 * DBA/2 * ICR

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cenpf^tm1Dbdr mutation (0 available); any Cenpf mutation (109 available)
Tg(Tnnt2-cre)5Blh mutation (1 available)

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

cardiovascular system

cardiovascular system phenotype ( J:185133 )

N	• development of heart structure is grossly normal

abnormal heart morphology ( J:185133 )

• modest reduction in coronary vasculature

abnormal myocardial fiber morphology ( J:185133 )

• costamere structure is impaired

• decreased number of myocytes in adults

abnormal intercalated disk morphology ( J:185133 )

• intercalated disc number is reduced 3.5 fold in mature ventricles

abnormal heart atrium morphology ( J:185133 )

• thinner walls

small heart ( J:185133 )

• smaller ventricles

myocardial trabeculae hypoplasia ( J:185133 )

• trabeculae are thinner and blunted

decreased heart weight ( J:185133 )

• weight at 4 days of age is 8.2mg compared to 10.5mg for controls

abnormal heart ventricle morphology ( J:185133 )

• smaller ventricles

dilated heart left ventricle ( J:185133 )

• internal dimension somewhat increased

thin ventricular wall ( J:185133 )

abnormal epicardium morphology ( J:185133 )

• modest reduction in epicardial thickness

cardiac fibrosis ( J:185133 )

• cardiac fibrosis in adults

abnormal cardiovascular system physiology ( J:185133 )

• decreasing ventricular function with age

dilated cardiomyopathy ( J:185133 )

• mice develop progressive dilated cardiomyopathy

decreased fetal cardiomyocyte proliferation ( J:185133 )

• three fold decrease in prenatal myocardiocyte proliferation

abnormal heart electrocardiography waveform feature ( J:185133 )

• at 3 months

prolonged PR interval ( J:185133 )

prolonged P wave ( J:185133 )

• P-P intervals greater than 200 ms

• slowing sinus rhythms

abnormal myocardial fiber physiology ( J:185133 )

• lower myocyte mitotic rates on days 1-4 after birth

growth/size/body

decreased body size ( J:185133 )

• normal to slightly smaller body size

mortality/aging

premature death ( J:185133 )

• 20% mortality in the 12 to 21 month age period

muscle

abnormal myocardial fiber morphology ( J:185133 )

• costamere structure is impaired

• decreased number of myocytes in adults

abnormal intercalated disk morphology ( J:185133 )

• intercalated disc number is reduced 3.5 fold in mature ventricles

myocardial trabeculae hypoplasia ( J:185133 )

• trabeculae are thinner and blunted

dilated cardiomyopathy ( J:185133 )

• mice develop progressive dilated cardiomyopathy

decreased fetal cardiomyocyte proliferation ( J:185133 )

• three fold decrease in prenatal myocardiocyte proliferation

cellular

decreased fetal cardiomyocyte proliferation ( J:185133 )

• three fold decrease in prenatal myocardiocyte proliferation

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
dilated cardiomyopathy		DOID:12930	OMIM:PS115200	J:185133

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