Phenotypes associated with this allele

• Allele Symbol: Tg(Nkx2-5-cre)9Eno
• Allele Name: transgene insertion 9, Eric N Olson
• Allele ID: MGI:3514028
• Summary: 13 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
cn1	Hand1^tm1Eno/Hand1^tm2Eno Rr133^tm1Eno/Rr133^+ Tg(Nkx2-5-cre)9Eno/0	either: (involves: 129S6/SvEvTac * Black Swiss) or (involves: 129S6/SvEvTac * C57BL/6)	MGI:3514076
cn2	Hand1^tm1Eno/Hand1^tm2Eno Rr133^tm1Eno/Rr133^tm1Eno Tg(Nkx2-5-cre)9Eno/0	either: (involves: 129S6/SvEvTac * Black Swiss) or (involves: 129S6/SvEvTac * C57BL/6)	MGI:3514077
cn3	Hand1^tm1Eno/Hand1^tm2Eno Tg(Nkx2-5-cre)9Eno/0	either: (involves: 129S6/SvEvTac * Black Swiss) or (involves: 129S6/SvEvTac * C57BL/6)	MGI:3514074
cn4	Hand2^tm1Dsr/Hand2^tm2.1Dsr Tg(Nkx2-5-cre)9Eno/0	involves: 129 * 129S7/SvEvBrd * C57BL/6	MGI:4940089
cn5	Gata4^tm1Grg/Gata4^tm1.1Wtp Tg(Nkx2-5-cre)9Eno/0	involves: 129 * C57BL/6	MGI:5427940
cn6	Syne1^tm1Chen/Syne1^tm1Chen Syne2^tm1.1Chen/Syne2^tm1.1Chen Tg(Nkx2-5-cre)9Eno/?	involves: 129S1/Sv * 129S4/SvJae * 129X1/SvJ * Black Swiss	MGI:5703835
cn7	Syne1^tm1Chen/Syne1^tm1Chen Tg(Nkx2-5-cre)9Eno/?	involves: 129S1/Sv * 129S4/SvJae * 129X1/SvJ * Black Swiss	MGI:5703836
cn8	Tmsb4x^tm1.2Chen/Y Tg(Nkx2-5-cre)9Eno/0	involves: 129S1/Sv * 129X1/SvJ * Black Swiss	MGI:5428018
cn9	Rac1^tm1Djk/Rac1^tm1Djk Tg(Nkx2-5-cre)9Eno/0	involves: 129S4/SvJae * BALB/c * C57BL/6	MGI:7545224
cn10	Hey2^tm1Eno/Hey2^tm2Eno Tg(Nkx2-5-cre)9Eno/0	involves: 129S6/SvEvTac * C57BL/6 * SJL	MGI:3711334
cn11	Zswim8^em2Jtm/Zswim8^em2Jtm Tg(Nkx2-5-cre)9Eno/0	involves: C57BL/6J	MGI:7537170
cn12	Commd9^tm1c(KOMP)Wtsi/Commd9^tm1c(KOMP)Wtsi Tg(Nkx2-5-cre)9Eno/0	involves: C57BL/6N	MGI:5796349
cn13	Irx3^tm3Hui/Irx3^tm3Hui Irx5^tm3Hui/Irx5^tm3Hui Tg(Nkx2-5-cre)9Eno/0	Not Specified	MGI:5444489

Genotype
MGI:3514076

cn1

• Allelic Composition: Hand1^tm1Eno/Hand1^tm2Eno; Rr133^tm1Eno/Rr133⁺; Tg(Nkx2-5-cre)9Eno/0
• Genetic Background: either: (involves: 129S6/SvEvTac * Black Swiss) or (involves: 129S6/SvEvTac * C57BL/6)

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

mortality/aging

embryonic lethality during organogenesis, complete penetrance ( J:94514 )

• no mutant embryos are found after E10.5

cardiovascular system

abnormal myocardial trabeculae morphology ( J:94514 )

• at E10.5 the myocardium is poorly trabeculated

thin myocardium ( J:94514 )

• at E10.5 the myocardium is thin

embryo

embryonic growth retardation ( J:94514 )

• at E10.5 mutant embryos appear slightly delayed compared to compound heterozygotes lacking only Hand1

muscle

abnormal myocardial trabeculae morphology ( J:94514 )

• at E10.5 the myocardium is poorly trabeculated

thin myocardium ( J:94514 )

• at E10.5 the myocardium is thin

growth/size/body

embryonic growth retardation ( J:94514 )

• at E10.5 mutant embryos appear slightly delayed compared to compound heterozygotes lacking only Hand1

Genotype
MGI:3514077

cn2

• Allelic Composition: Hand1^tm1Eno/Hand1^tm2Eno; Rr133^tm1Eno/Rr133^tm1Eno; Tg(Nkx2-5-cre)9Eno/0
• Genetic Background: either: (involves: 129S6/SvEvTac * Black Swiss) or (involves: 129S6/SvEvTac * C57BL/6)

mortality/aging

embryonic lethality between somite formation and embryo turning, complete penetrance ( J:94514 )

• no mutant embryos are found after E9.0

cardiovascular system

abnormal myocardium layer morphology ( J:94514 )

• at E9.0 some segments of the myocardial wall contain cells that resemble mesenchymal cells rather than cardiomyocytes

abnormal heart atrium morphology ( J:94514 )

• at E9.0 only a single atrium is present

abnormal heart ventricle morphology ( J:94514 )

• at E9.0 only a single immature ventricle is present and the lumen of the ventricle is abnormally narrow

trabecula carnea hypoplasia ( J:94514 )

muscle

abnormal myocardium layer morphology ( J:94514 )

• at E9.0 some segments of the myocardial wall contain cells that resemble mesenchymal cells rather than cardiomyocytes

trabecula carnea hypoplasia ( J:94514 )

Genotype
MGI:3514074

cn3

• Allelic Composition: Hand1^tm1Eno/Hand1^tm2Eno; Tg(Nkx2-5-cre)9Eno/0
• Genetic Background: either: (involves: 129S6/SvEvTac * Black Swiss) or (involves: 129S6/SvEvTac * C57BL/6)

mortality/aging

postnatal lethality, complete penetrance ( J:94514 )

• most mutants die within 2-4 days of birth

cardiovascular system

abnormal heart morphology ( J:94514 )

• heart defects similar to those seen in compound heterozygotes hemizygous for Tg(Myhca-cre)2182Mds are found

Genotype
MGI:4940089

cn4

• Allelic Composition: Hand2^tm1Dsr/Hand2^tm2.1Dsr; Tg(Nkx2-5-cre)9Eno/0
• Genetic Background: involves: 129 * 129S7/SvEvBrd * C57BL/6

mortality/aging

embryonic lethality during organogenesis, complete penetrance ( J:169213 )

• mice die at E12.5

cardiovascular system

decreased heart left ventricle size ( J:169213 )

• slightly at E9.5

decreased heart right ventricle size ( J:169213 )

• slightly at E9.5

Genotype
MGI:5427940

cn5

• Allelic Composition: Gata4^tm1Grg/Gata4^tm1.1Wtp; Tg(Nkx2-5-cre)9Eno/0
• Genetic Background: involves: 129 * C57BL/6

Growth retardation and myocardial thinning in Gata4^tm1.1Wtp/Gata4^tm1Grg Tg(Nkx2-5-cre)9Eno/0 mice

mortality/aging

embryonic lethality during organogenesis, incomplete penetrance ( J:185124 )

• at E10.5

growth/size/body

embryonic growth retardation ( J:185124 )

• severe

cardiovascular system

thin myocardium ( J:185124 )

embryo

embryonic growth retardation ( J:185124 )

• severe

muscle

thin myocardium ( J:185124 )

Genotype
MGI:5703835

cn6

• Allelic Composition: Syne1^tm1Chen/Syne1^tm1Chen; Syne2^tm1.1Chen/Syne2^tm1.1Chen; Tg(Nkx2-5-cre)9Eno/?
• Genetic Background: involves: 129S1/Sv * 129S4/SvJae * 129X1/SvJ * Black Swiss

Ultrastructural nuclear abnormalities in Syne1^tm1Chen/Syne1^tm1Chen Syne2^tm1.1ChenSyne2^tm1.1Chen Tg(Nkx2-5-cre)9Eno/? cardiomyocytes

cardiovascular system

cardiovascular system phenotype ( J:211007 )

N • heart rate remains unchanged through course of study

abnormal heart morphology ( J:211007 )

abnormal heart weight ( J:211007 )

• significant increase in heart weight/body weight and heart weight/total length ratios at 10 weeks of age

thin ventricular wall ( J:211007 )

• wall thinning observed at 10 weeks but not at 5 weeks of age

• thinning continues through 25 and 52 weeks

cardiac fibrosis ( J:211007 )

• at 10 weeks

abnormal cardiovascular system physiology ( J:211007 )

decreased cardiac muscle contractility ( J:211007 )

• diminished fractional shortening at 10 weeks but not 5 weeks of age

• fractional shortening continues to decline at 25 and 52 weeks

increased myocardial fiber apoptosis ( J:211007 )

• at 10 weeks of age

cellular

increased myocardial fiber apoptosis ( J:211007 )

• at 10 weeks of age

abnormal cell nucleus morphology ( J:211007 )

• nuclei are considerably enlarged and binuclei are closer together

• nuclei are less circular

• nuclear abnormalities begin to appear at 1 day of age

muscle

decreased cardiac muscle contractility ( J:211007 )

• diminished fractional shortening at 10 weeks but not 5 weeks of age

• fractional shortening continues to decline at 25 and 52 weeks

increased myocardial fiber apoptosis ( J:211007 )

• at 10 weeks of age

Genotype
MGI:5703836

cn7

• Allelic Composition: Syne1^tm1Chen/Syne1^tm1Chen; Tg(Nkx2-5-cre)9Eno/?
• Genetic Background: involves: 129S1/Sv * 129S4/SvJae * 129X1/SvJ * Black Swiss

cellular

abnormal cell nucleus morphology ( J:211007 )

• nuclei are less circular

• nuclei are closer together in binuclear cardiomyocytes

abnormal cell nucleus size ( J:211007 )

• nuclei are enlarged

Genotype
MGI:5428018

cn8

• Allelic Composition: Tmsb4x^tm1.2Chen/Y; Tg(Nkx2-5-cre)9Eno/0
• Genetic Background: involves: 129S1/Sv * 129X1/SvJ * Black Swiss

normal phenotype

no abnormal phenotype detected ( J:185264 )

♂ • mutant mice are viable to adulthood

♂ • mice did not have alterations in heart weight/body weight or heart weight/ tibia length compared with control mice

♂ • histological observations as well as expression of fetal gene and fibrotic markers were also similar to control mice

♂ • mice have normal cardiac function, chamber size, and wall thickness compared with their littermate controls

♂ • no changes in vessel volume in mutant mice

Genotype
MGI:7545224

cn9

• Allelic Composition: Rac1^tm1Djk/Rac1^tm1Djk; Tg(Nkx2-5-cre)9Eno/0
• Genetic Background: involves: 129S4/SvJae * BALB/c * C57BL/6

mortality/aging

perinatal lethality, complete penetrance ( J:321017 )

• although mice are alive from E11.5 to E18.5, all neonates are found dead at P0

cardiovascular system

abnormal cardiac outflow tract development ( J:321017 )

• at E14.5-P0, hearts exhibit impaired alignment of the outflow tract (OFT) to the ventricles

abnormal heart morphology ( J:321017 )

• at E14.5-P0, all (17 of 17) hearts display more than one type of congenital heart defect (CHD), not observed in control hearts

abnormal myocardium compact layer morphology ( J:321017 )

• at E15.5, the compact myocardium of both ventricles is poorly formed

thin myocardium compact layer ( J:321017 )

• at E14.5-P0, all hearts (17 of 17) exhibit a thin compact myocardium

thin ventricle myocardium compact layer ( J:321017 )

• at E15.5, both the left ventricle (LV) and right ventricle (RV) show a significant decrease in compact myocardium thickness relative to controls

abnormal ventricle myocardium morphology ( J:321017 )

• at E18.5, the ventricular myocardium shows severe disruption of F-actin filament organization

• at E9.5, the cell proliferation rate in the ventricular myocardium is significantly lower than in control hearts, as assessed by the percentage of pHH3+ and cyclin D1+ cardiomyocytes

• however, no aberrant apoptosis is detected in the ventricular myocardium at E9.5

abnormal trabecula carnea morphology ( J:321017 )

• at E15.5, both the left ventricle (LV) and right ventricle (RV) show hypertrabeculation; the trabecular to compact myocardium ratio is increased by >2.5-fold relative to controls

abnormal heart development ( J:321017 )

• at E12.5, mRNA expression of Scrib and other cardiac transcription and growth factors (Nkx2.5, Gata4, Tbx5, Tbx20, Hand1, Hand2 and Bmp10) is significantly decreased relative to control hearts

abnormal fetal cardiomyocyte morphology ( J:321017 )

• at E18.5, cardiomyocytes in the ventricular myocardium exhibit a rounded morphology and appear highly disorganized in both the RV and LV while the short axis of cardiomyocyte diameter is significantly larger than in controls, indicating impaired cardiomyocyte polarization and elongation

• at E15.5, overall expression of SCRIB (scribble planar cell polarity protein) is significantly reduced in the myocardium surrounding the aorta (OFT), RV and LV, supporting a failure of cardiomyocytes to undergo polarization

double outlet right ventricle ( J:321017 )

• at E14.5-P0, 64.7% (11 of 17) hearts exhibit DORV: both pulmonary artery and aorta are connected to the right ventricle

overriding aortic valve ( J:321017 )

• at E14.5-P0, 35.3% (6 of 17) hearts exhibit and overriding aorta

abnormal heart apex morphology ( J:321017 )

• at E14.5-P0, all (17 of 17) hearts show a bifid cardiac apex (a bifurcation between the right and the left ventricle)

ventricular septal defect ( J:321017 )

• at E14.5-P0, all hearts (17 of 17) show ventricular septal defects

decreased fetal cardiomyocyte proliferation ( J:321017 )

• at E9.5, the cell proliferation rate in the ventricular myocardium is significantly lower than in control hearts, as assessed by the percentage of pHH3+ and cyclin D1+ cardiomyocytes

cellular

decreased fetal cardiomyocyte proliferation ( J:321017 )

• at E9.5, the cell proliferation rate in the ventricular myocardium is significantly lower than in control hearts, as assessed by the percentage of pHH3+ and cyclin D1+ cardiomyocytes

muscle

abnormal myocardium compact layer morphology ( J:321017 )

• at E15.5, the compact myocardium of both ventricles is poorly formed

thin myocardium compact layer ( J:321017 )

• at E14.5-P0, all hearts (17 of 17) exhibit a thin compact myocardium

thin ventricle myocardium compact layer ( J:321017 )

• at E15.5, both the left ventricle (LV) and right ventricle (RV) show a significant decrease in compact myocardium thickness relative to controls

abnormal ventricle myocardium morphology ( J:321017 )

• at E18.5, the ventricular myocardium shows severe disruption of F-actin filament organization

• at E9.5, the cell proliferation rate in the ventricular myocardium is significantly lower than in control hearts, as assessed by the percentage of pHH3+ and cyclin D1+ cardiomyocytes

• however, no aberrant apoptosis is detected in the ventricular myocardium at E9.5

abnormal trabecula carnea morphology ( J:321017 )

• at E15.5, both the left ventricle (LV) and right ventricle (RV) show hypertrabeculation; the trabecular to compact myocardium ratio is increased by >2.5-fold relative to controls

decreased fetal cardiomyocyte proliferation ( J:321017 )

• at E9.5, the cell proliferation rate in the ventricular myocardium is significantly lower than in control hearts, as assessed by the percentage of pHH3+ and cyclin D1+ cardiomyocytes

Genotype
MGI:3711334

cn10

• Allelic Composition: Hey2^tm1Eno/Hey2^tm2Eno; Tg(Nkx2-5-cre)9Eno/0
• Genetic Background: involves: 129S6/SvEvTac * C57BL/6 * SJL

cardiovascular system

cardiovascular system phenotype ( J:121577 )

N

enlarged heart ( J:121577 )

• adult mice have grossly enlarged hearts

abnormal heart ventricle morphology ( J:121577 )

• base of dilated right ventricle merges with interventricular septum above apex of heart; this is apparent by E13.5 and persists in adults

• at 6 weeks of age, ectopic activation of atrial regular and structural genes (>10-fold greater than wild-type littermates) is observed in ventricular myocardium

abnormal interventricular septum morphology ( J:121577 )

• VSDs are not observed in all mice, however, some show VSDs or delay of ventricular septal formation at E13.5-17.5

abnormal heart left ventricle morphology ( J:121577 )

• at 6 weeks of age, mice show an increase in systolic left ventricular internal diameter (LVID)

• normal diastolic left ventricular internal diameter does not change significantly relative to wild-type

• atrial naturietic factor (ANF) and Tbx5 expression is increased in left ventricle at E17.5, whereas expression is seen in atria and trabecular cells in wild-type

dilated heart right ventricle ( J:121577 )

decreased cardiac muscle contractility ( J:121577 )

• deterioration in heart contractility, accompanied by decreased fractional shortening (FS)

muscle

decreased cardiac muscle contractility ( J:121577 )

• deterioration in heart contractility, accompanied by decreased fractional shortening (FS)

growth/size/body

enlarged heart ( J:121577 )

• adult mice have grossly enlarged hearts

Genotype
MGI:7537170

cn11

• Allelic Composition: Zswim8^em2Jtm/Zswim8^em2Jtm; Tg(Nkx2-5-cre)9Eno/0
• Genetic Background: involves: C57BL/6J

mortality/aging

mortality/aging ( J:338912 )

N • unlike germline null mice, conditional loss in hear progenitor cells does not result i neonatal lethality

cardiovascular system

globular heart ( J:338912 )

• apex is less distinct

• cardiac phenotypes are less severe than in germline null mice

dilated heart ventricle ( J:338912 )

• dilated ventricles

Genotype
MGI:5796349

cn12

• Allelic Composition: Commd9^{tm1c(KOMP)Wtsi}/Commd9^{tm1c(KOMP)Wtsi}; Tg(Nkx2-5-cre)9Eno/0
• Genetic Background: involves: C57BL/6N
• Cell Lines: EPD0136_6_D10

mortality/aging

prenatal lethality, incomplete penetrance ( J:230747 )

• only 14% (versus expected 25%) of mice are viable at birth; time of lethality is not specified

Genotype
MGI:5444489

cn13

• Allelic Composition: Irx3^tm3Hui/Irx3^tm3Hui; Irx5^tm3Hui/Irx5^tm3Hui; Tg(Nkx2-5-cre)9Eno/0
• Genetic Background: Not Specified

cardiovascular system

cardiovascular system phenotype ( J:189007 )

N • no defects in heart morphology are detected