About   Help   FAQ
Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Tg(Myh6-cre)2182Mds
transgene insertion 2182, Michael D Schneider
MGI:2386742
Summary 80 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
cn1
Ctnnb1tm2(Nfkbia)Rsu/Ctnnb1+
Tg(Myh6-cre)2182Mds/0
B6.Cg-Ctnnb1tm2(Nfkbia)Rsu Tg(Myh6-cre)2182Mds MGI:3706583
cn2
Hand1tm1Eno/Hand1tm2Eno
Tg(Myh6-cre)2182Mds/0
either: (involves: 129S6/SvEvTac * Black Swiss) or (involves: 129S6/SvEvTac * C57BL/6) MGI:3514054
cn3
Rb1tm2Brn/Rb1tm2Brn
Rbl2tm1Tyj/Rbl2tm1Tyj
Tg(Myh6-cre)2182Mds/Tg(Myh6-cre)2182Mds
involves: 129 * 129S2/SvPas * FVB/N MGI:3710679
cn4
Cxadrtm1Know/Cxadrtm1Know
Tg(Myh6-cre)2182Mds/0
involves: 129 * Black Swiss * FVB/N MGI:3815088
cn5
Gata4tm1Grg/Gata4tm1.1Wtp
Tg(Myh6-cre)2182Mds/0
involves: 129 * C57BL/6 * FVB/N MGI:5427939
cn6
Juptm1.1Shou/Juptm1.1Shou
Tg(Myh6-cre)2182Mds/0
involves: 129 * C57BL/6J MGI:5296512
cn7
Rhebtm1.1Otsu/Rhebtm1.1Otsu
Tg(Myh6-cre)2182Mds/0
involves: 129 * C57BL/6J * FVB/N MGI:5502747
cn8
Eif4ebp1tm1Nso/Eif4ebp1tm1Nso
Rhebtm1.1Otsu/Rhebtm1.1Otsu
Tg(Myh6-cre)2182Mds/0
involves: 129 * C57BL/6J * FVB/N MGI:5502748
cn9
Rb1tm2Brn/Rb1tm2Brn
Tg(Myh6-cre)2182Mds/Tg(Myh6-cre)2182Mds
involves: 129 * FVB MGI:3710677
cn10
Hprttm2(CAG-TBX2,-EGFP)Akis/Hprt+
Tg(Myh6-cre)2182Mds/0
involves: 129 * FVB/N * NMRI MGI:5314543
cn11
Srftm1Rjs/Srftm2.1Nor
Tg(Myh6-cre)2182Mds/0
involves: 129P2/OlaHsd * 129S7/SvEvBrd * C57BL/6 * FVB/N MGI:3608600
cn12
Gja1tm10Kwi/Gja1tm10Kwi
Tg(Myh6-cre)2182Mds/0
involves: 129P2/OlaHsd * C57BL/6 * FVB/N MGI:5477915
cn13
Gjc1tm1.1(Gjd2)Kwi/Gjc1tm1.1(Gjd2)Kwi
Tg(Myh6-cre)2182Mds/0
involves: 129P2/OlaHsd * C57BL/6 * FVB/N * SJL MGI:5317174
cn14
Npr1tm1Kuhn/Npr1tm1Kuhn
Tg(Myh6-cre)2182Mds/0
involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * FVB/N MGI:3687001
cn15
Gab1tm1Nmoc/Gab1tm1Nmoc
Gab2tm1Thir/Gab2tm1Thir
Tg(Myh6-cre)2182Mds/?
involves: 129S1/Sv * 129X1/SvJ * C57BL/6J * FVB/N MGI:3721269
cn16
Cxadrtm1Mds/Cxadrtm1Mds
Tg(Myh6-cre)2182Mds/0
involves: 129S1/Sv * 129X1/SvJ * FVB/N MGI:3711507
cn17
Cxadrtm1Mds/Cxadrtm1.1Mds
Tg(Myh6-cre)2182Mds/0
involves: 129S1/Sv * 129X1/SvJ * FVB/N MGI:3711512
cn18
Acvr1tm1Vk/Acvr1tm1.1Vk
Tg(Myh6-cre)2182Mds/0
involves: 129S1/Sv * 129X1/SvJ * FVB/N MGI:3697609
cn19
Shc1tm8Paw/Shc1tm9.1Paw
Tg(Myh6-cre)2182Mds/0
involves: 129S1/Sv * 129X1/SvJ * FVB/N MGI:3717096
cn20
Stat3tm1Xyfu/Stat3+
Tg(Myh6-cre)2182Mds/0
involves: 129S1/Sv * FVB/N MGI:4879021
cn21
Stat3tm1Xyfu/Stat3tm1.1Xyfu
Tg(Myh6-cre)2182Mds/0
involves: 129S1/Sv * FVB/N MGI:4879018
cn22
Mdm2tm1Glo/Mdm2tm2.1Glo
Tg(Myh6-cre)2182Mds/0
Trp53tm1Tyj/Trp53tm1Tyj
involves: 129S2/SvPas * 129S7/SvEvBrd * FVB/N MGI:3616710
cn23
Gja1tm8Kwi/Gja1+
Tg(Myh6-cre)2182Mds/0
involves: 129S2/SvPas * FVB/N MGI:3807709
cn24
Fstl1tm1.1Mvdh/Fstl1tm1.1Mvdh
Tg(Myh6-cre)2182Mds/0
involves: 129S4/SvJae * C57BL/6 * FVB/N MGI:5297552
cn25
Vhltm1Jae/Vhltm1Jae
Tg(Myh6-cre)2182Mds/0
involves: 129S4/SvJae * FVB/N MGI:5304714
cn26
Mef2ctm1Eno/Mef2ctm1Jjs
Tg(Myh6-cre)2182Mds/0
involves: 129S4/SvJaeSor * 129S6/SvEvTac * 129S7/SvEvBrd * FVB/N MGI:3613580
cn27
Grk5tm2Rjl/Grk5tm2Rjl
Tg(Myh6-cre)2182Mds/0
involves: 129S4/SvJaeSor * C57BL/6J * FVB/N MGI:5582621
cn28
Mdm4tm2Glo/Mdm4tm2.1Glo
Tg(Myh6-cre)2182Mds/0
involves: 129S4/SvJaeSor * C57BL/6J * FVB/N MGI:3616711
cn29
Ptpn11tm6Bgn/Ptpn11+
Tg(Myh6-cre)2182Mds/0
involves: 129S6/SvEvTac * C57BL/6 * FVB/N MGI:3840255
cn30
Ednratm2Ywa/Ednratm2Ywa
Tg(Myh6-cre)2182Mds/0
involves: 129S6/SvEvTac * C57BL/6J * FVB/N MGI:2687389
cn31
Myh10tm7Rsad/Myh10tm7Rsad
Tg(Myh6-cre)2182Mds/0
involves: 129S6/SvEvTac * C57BL/6J * FVB/N MGI:4946095
cn32
Egln1tm1Kael/Egln1tm1Kael
Tg(Myh6-cre)2182Mds/0
involves: 129S6/SvEvTac * FVB/N MGI:5304715
cn33
Edn1tm1Ywa/Edn1tm1Ywa
Tg(Myh6-cre)2182Mds/0
involves: 129S6/SvEvTac * FVB/N MGI:3044594
cn34
Gata4tm2(Gata4)Wtp/Gata4+
Tg(Myh6-cre)2182Mds/0
involves: 129S6/SvEvTac * FVB/N MGI:3802690
cn35
Egln1tm1Kael/Egln1tm1Kael
Egln3tm1Vlcg/Egln3tm1Vlcg
Tg(Myh6-cre)2182Mds/0
involves: 129S6/SvEvTac * FVB/N MGI:5304713
cn36
Gt(ROSA)26Sortm4(HIF2A*)Kael/Gt(ROSA)26Sor+
Tg(Myh6-cre)2182Mds/0
involves: 129S6/SvEvTac * FVB/N MGI:5304716
cn37
Tbx1tm1Bld/Tbx1tm3Bld
Tg(Myh6-cre)2182Mds/0
involves: 129S7/SvEvBrd * C57BL/6 MGI:3046803
cn38
Rassf1tm1.1Brd/Rassf1tm1.1Brd
Tg(Myh6-cre)2182Mds/0
involves: 129S7/SvEvBrd * FVB/N MGI:4837486
cn39
Fkbp1atm1.1Shou/Fkbp1atm1Zuk
Tg(Myh6-cre)2182Mds/0
involves: 129S7/SvEvBrd * FVB/N MGI:5490257
cn40
Mdm2tm1Glo/Mdm2tm2.1Glo
Tg(Myh6-cre)2182Mds/0
involves: 129S7/SvEvBrd * FVB/N MGI:3616709
cn41
Hdac3tm1.1Eno/Hdac3tm1.1Eno
Tg(Myh6-cre)2182Mds/?
involves: 129S/SvEv * C57BL/6 MGI:3831703
cn42
Hdac1tm1.1Eno/Hdac1tm1.1Eno
Tg(Myh6-cre)2182Mds/?
involves: 129S/SvEv * C57BL/6 * CD-1 * FVB/N MGI:3718385
cn43
Hdac1tm1.1Eno/Hdac1tm1.1Eno
Hdac2tm1.1Eno/Hdac2tm1.1Eno
Tg(Myh6-cre)2182Mds/?
involves: 129S/SvEv * C57BL/6 * CD-1 * FVB/N MGI:3718388
cn44
Hdac2tm1.1Eno/Hdac2tm1.1Eno
Tg(Myh6-cre)2182Mds/?
involves: 129S/SvEv * C57BL/6 * CD-1 * FVB/N MGI:3718387
cn45
Atg5tm1Myok/Atg5tm1Myok
Tg(Myh6-cre)2182Mds/?
involves: 129S/SvEv * C57BL/6 * FVB/N MGI:3713114
cn46
Prkd1tm1Eno/Prkd1tm1Eno
Tg(Myh6-cre)2182Mds/?
involves: 129S/SvEv * C57BL/6 * FVB/N MGI:3778390
cn47
Atg5tm1Myok/Atg5tm1Myok
Rhebtm1.1Otsu/Rhebtm1.1Otsu
Tg(Myh6-cre)2182Mds/0
involves: 129S/SvEv * C57BL/6J * FVB/N MGI:5502749
cn48
Wwtr1tm1.1Eno/Wwtr1tm1.1Eno
Tg(Myh6-cre)2182Mds/0
involves: 129S/SvEv * FVB/N MGI:5544299
cn49
Yap1tm1.1Eno/Yap1tm1.1Eno
Tg(Myh6-cre)2182Mds/0
involves: 129S/SvEv * FVB/N MGI:5544295
cn50
Wwtr1tm1.1Eno/Wwtr1tm1.1Eno
Yap1tm1.1Eno/Yap1+
Tg(Myh6-cre)2182Mds/0
involves: 129S/SvEv * FVB/N MGI:5544296
cn51
Wwtr1tm1.1Eno/Wwtr1+
Yap1tm1.1Eno/Yap1tm1.1Eno
Tg(Myh6-cre)2182Mds/0
involves: 129S/SvEv * FVB/N MGI:5544297
cn52
Wwtr1tm1.1Eno/Wwtr1tm1.1Eno
Yap1tm1.1Eno/Yap1tm1.1Eno
Tg(Myh6-cre)2182Mds/0
involves: 129S/SvEv * FVB/N MGI:5544298
cn53
Rock2tm2Liao/Rock2tm2Liao
Tg(Myh6-cre)2182Mds/?
involves: 129/Sv * C57BL/6 MGI:5491820
cn54
Jarid2tm1Yskl/Jarid2tm1Yskl
Tg(Myh6-cre)2182Mds/0
involves: 129/Sv * FVB/N MGI:3689723
cn55
Ppargc1atm1Dpk/Ppargc1atm1Dpk
Ppargc1btm1Dpk/Ppargc1btm1Dpk
Tg(Myh6-cre)2182Mds/0
involves: 129X1/SvJ * FVB/N MGI:3802663
cn56
Rce1tm2Kim/Rce1tm2.1Kim
Tg(Myh6-cre)2182Mds/0
involves: C57BL/6 MGI:3032514
cn57
Abl1tm1Goff/Abl1tm1Goff
Tg(Myh6-cre)2182Mds/0
involves: C57BL/6 MGI:4421539
cn58
Lpltm1Ijg/Lpltm1Ijg
Tg(Myh6-cre)2182Mds/?
involves: C57BL/6 MGI:3045423
cn59
Nox4tm1.1Jusa/Nox4tm1.1Jusa
Tg(Myh6-cre)2182Mds/0
involves: C57BL/6 * FVB MGI:4830886
cn60
Nr3c1tm1.1Jaci/Nr3c1tm1.1Jaci
Tg(Myh6-cre)2182Mds/0
involves: C57BL/6 * FVB/N MGI:5526029
cn61
Atp6ap2tm1.1Aich/Y
Tg(Myh6-cre)2182Mds/?
involves: C57BL/6 * FVB/N MGI:4836055
cn62
Rbm20tm2.1Hgra/Rbm20+
Tg(Myh6-cre)2182Mds/0
involves: C57BL/6 * FVB/N MGI:5566535
cn63
Klf5tm2.1Rng/Klf5tm2.1Rng
Tg(Myh6-cre)2182Mds/0
involves: C57BL/6 * FVB/N MGI:4421818
cn64
Hey2tm2.1Mtc/Hey2tm2.1Mtc
Tg(Myh6-cre)2182Mds/0
involves: C57BL/6 * FVB/N * SJL MGI:5433520
cn65
Mapk14tm1.2Otsu/Mapk14tm1.2Otsu
Tg(Myh6-cre)2182Mds/0
involves: C57BL/6J MGI:3525858
cn66
Raf1tm2Bacc/Raf1tm2Bacc
Tg(Myh6-cre)2182Mds/0
involves: C57BL/6J MGI:3510547
cn67
Map3k5tm1Hijo/Map3k5tm1Hijo
Raf1tm2Bacc/Raf1tm2Bacc
Tg(Myh6-cre)2182Mds/0
involves: C57BL/6J MGI:3510548
cn68
Capns1tm1.1Otsu/Capns1tm1.1Otsu
Tg(Myh6-cre)2182Mds/0
involves: C57BL/6J * FVB/N MGI:5293311
cn69
Fkbp1atm1.1Shou/Fkbp1atm1.1Shou
Tg(Myh6-cre)2182Mds/0
involves: FVB/N MGI:5319195
cn70
Cxadrtm1Mgot/Cxadrtm1Mgot
Tg(Myh6-cre)2182Mds/0
involves: FVB/N MGI:3812382
cn71
Map3k7tm1Mds/Map3k7tm1Mds
Tg(Myh6-cre)2182Mds/0
involves: FVB/N MGI:3696414
cn72
Tg(CAG-Map3k7*K63W)1232Mds/0
Tg(Myh6-cre)2182Mds/0
involves: FVB/N MGI:4461321
cn73
Lpltm1Ijg/Lpltm1Ijg
Tg(Myh6-cre)2182Mds/0
involves: FVB/N MGI:3655840
cn74
Gata4tm1.1Wtp/Gata4tm1.1Wtp
Tg(Myh6-cre)2182Mds/0
involves: FVB/N MGI:3639277
cn75
Tg(CAG-cat,-TBX3)1Vmc/0
Tg(Myh6-cre)2182Mds/0
involves: FVB/N MGI:5314604
cn76
Ehmt1tm2Yshk/Ehmt1tm2Yshk
Tg(Myh6-cre)2182Mds/0
involves: FVB/N MGI:5543777
cn77
Gja1tm1Gfi/Gja1tm1Gfi
Tg(Myh6-cre)2182Mds/0
Not Specified MGI:3051890
cn78
Ttntm1Her/Ttntm1Her
Tg(Myh6-cre)2182Mds/0
Not Specified MGI:2651646
cx79
Med13tm1Eno/Med13tm1Eno
Tg(Myh6-cre)2182Mds/0
involves: 129 * C57BL/6 * FVB/N MGI:5431523
tg80
Tg(Myh6-cre)2182Mds/0 Not Specified MGI:5526034


Genotype
MGI:3706583
cn1
Allelic
Composition
Ctnnb1tm2(Nfkbia)Rsu/Ctnnb1+
Tg(Myh6-cre)2182Mds/0
Genetic
Background
B6.Cg-Ctnnb1tm2(Nfkbia)Rsu Tg(Myh6-cre)2182Mds
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ctnnb1tm2(Nfkbia)Rsu mutation (0 available); any Ctnnb1 mutation (27 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• mutants display an attenuated hypertrophic response compared with controls upon infusion of angiotensin II or isoproterenol as well as reduced expression of hypertrophy markers

homeostasis/metabolism
• mutants display an attenuated hypertrophic response compared with controls upon infusion of angiotensin II or isoproterenol as well as reduced expression of hypertrophy markers




Genotype
MGI:3514054
cn2
Allelic
Composition
Hand1tm1Eno/Hand1tm2Eno
Tg(Myh6-cre)2182Mds/0
Genetic
Background
either: (involves: 129S6/SvEvTac * Black Swiss) or (involves: 129S6/SvEvTac * C57BL/6)
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Hand1tm1Eno mutation (1 available); any Hand1 mutation (3 available)
Hand1tm2Eno mutation (0 available); any Hand1 mutation (3 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• most mutants die within 3 days of birth with only 4% surviving to P10 and less than 2% reaching adulthood

cardiovascular system
• immature endocardial cushions are seen in mutant hearts at E13.5
• hyperplastic atrioventicular valves are seen in all mutants and these valves are thickened in neonates
• 90% of mutants have membranous ventricular septal defects and these defects can be detected as early as E10.5
• the muscular ventricular septum is thickened and disorganized
• at E11.5 and E13.5 the left ventricle is reduced in size, however the size is normal at birth

homeostasis/metabolism
• most mutants become cyanotic within 3 days of birth




Genotype
MGI:3710679
cn3
Allelic
Composition
Rb1tm2Brn/Rb1tm2Brn
Rbl2tm1Tyj/Rbl2tm1Tyj
Tg(Myh6-cre)2182Mds/Tg(Myh6-cre)2182Mds
Genetic
Background
involves: 129 * 129S2/SvPas * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Rb1tm2Brn mutation (4 available); any Rb1 mutation (61 available)
Rbl2tm1Tyj mutation (1 available); any Rbl2 mutation (22 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• 36% die suddenly by 12 weeks of age and only 48% survive to 6 months of age

cardiovascular system
• cardiomyocytes have an increase in diameter and contain large, hyperchromatic nuclei
• mutants exhibit a 3-fold increase in the heart weight-to-body weight ratio at 8 weeks of age but not in the neonatal time period
• mutants surviving to 12 weeks of age show evidence of LV dilation
• end-diastolic diameter and end-systolic diameter are increased and left ventricular fractional shortening and circumferential shortening velocity are decreased in mutants surviving to 12 seeks of age, indicating left ventricular systolic dysfunction
• seen in mutants surviving to 12 weeks of age
• hearts exhibit persistent myocyte cycling

respiratory system
• 36% develop signs of respiratory distress

homeostasis/metabolism
• 36% develop signs of generalized edema

muscle
• cardiomyocytes have an increase in diameter and contain large, hyperchromatic nuclei
• end-diastolic diameter and end-systolic diameter are increased and left ventricular fractional shortening and circumferential shortening velocity are decreased in mutants surviving to 12 seeks of age, indicating left ventricular systolic dysfunction




Genotype
MGI:3815088
cn4
Allelic
Composition
Cxadrtm1Know/Cxadrtm1Know
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: 129 * Black Swiss * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cxadrtm1Know mutation (0 available); any Cxadr mutation (6 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• 8-week old mice have a clear decrease in ZO-1 localized to the intercalated disc
• intercalated discs are punctuated by areas where there is an increase in the space between cardiac myocytes, usually near the adherens junctions
• disorganization of the electron-dense structures associated with the intercalated disc is observed
• disorganization of the myofilaments that attach to the intercalated disc also occurs
• cardiac fibrosis occurs in mice 25 weeks of age
• there is a progressive decrease in fractional shortening at 21 and 25 weeks of age
• fractional shortening degrades from a normal level at 17 weeks of age to half that of wild-type by 25 weeks of age
• AV-node block occurs in these mice with half the mice having a complete AV block and the other half having first-degree AV block over 90% of the time
• in mice without complete AV blockage, there is an extended PR interval
• mean PR interval is 36.5 ms in wild-type compared with 53.9 ms in mutant mice
• an averaged P-wave is not detectable because of the dissociation between the atrial and the ventricular depolarizations
• the R-to-R intervals are shorter in these mice with 127.3 ms in wild-type mice vs. 97.4 ms in mutant mice

muscle
• 8-week old mice have a clear decrease in ZO-1 localized to the intercalated disc
• intercalated discs are punctuated by areas where there is an increase in the space between cardiac myocytes, usually near the adherens junctions
• disorganization of the electron-dense structures associated with the intercalated disc is observed
• disorganization of the myofilaments that attach to the intercalated disc also occurs
• there is a progressive decrease in fractional shortening at 21 and 25 weeks of age
• fractional shortening degrades from a normal level at 17 weeks of age to half that of wild-type by 25 weeks of age




Genotype
MGI:5427939
cn5
Allelic
Composition
Gata4tm1Grg/Gata4tm1.1Wtp
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: 129 * C57BL/6 * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gata4tm1.1Wtp mutation (0 available); any Gata4 mutation (15 available)
Gata4tm1Grg mutation (1 available); any Gata4 mutation (15 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype

Hearts of Gata4tm1.1Wtp/Gata4tm1Grg Tg(Myh6-cre)2182Mds/0 mice appear normal

normal phenotype
• viable with no signs of embryonic growth retardation or myocardial thinning




Genotype
MGI:5296512
cn6
Allelic
Composition
Juptm1.1Shou/Juptm1.1Shou
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: 129 * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Juptm1.1Shou mutation (0 available); any Jup mutation (139 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• mutants suddenly die starting at 1 month of age, with an average life-span of 4.6 months

cardiovascular system
• mutants exhibit severe liver congestion
• cardiomyocytes are hypertrophic, showing an increase in cross-sectional area
• hearts show loss of desmosomes, however adherens junctions are normal
• myocardial calcification is seen in 2-month old ventricles
• heart is enlarged at 2 months of age
• hearts exhibit regional dysplasia of ventricular walls and ventricular aneurysms
• cardiac fibrosis is seen in atria and ventricles; fibrosis ranges from moderate to severe
• ECG shows compromised systolic function of the left ventricle
• fragmented diastolic potentials are seen in right ventricle
• hearts show reduced fractional shortening and ejection fraction
• at 1 and 2 months of age, mutants exhibit spontaneous non-sustained ventricular tachycardia
• sustained monomorphic ventricular tachycardias are induced in mutants by bust or programmed ventricular stimulation in 6 of 7 mutants but not in controls
• mutants exhibit intracardiac bipolar electrograms during ventricular tachycardia
• in Langendorff-perfused hearts, conduction velocity max and min are reduced
• at 1 month of age, mutants exhibit complex electrophysiological abnormalities, with low amplitude of the QRS complex, prolonged PR interval and spontaneous non-sustained ventricular tachycardia
• by 2 months of age, the amplitude of the P-wave is higher, amplitude of QRS complex is lower, the PR interval is prolonged, and frequent spontaneous ventricular ectopic beats are seen
• at 1 and 2 months of age, mutants exhibit prolonged PR interval
• by 2 months of age, the amplitude of the P-wave is higher
• ECG shows decreased QRS complex amplitude at P18, 1 month and 2 months of age
• 18 day old mutants exhibit extensive cardiomyocyte death, including apoptisis and necrosis
• mutants show rapid and progressive development of cardiomyopathy; hearts appear normal at P12 but by P18, show signs of fibrosis and heart enlargement by 1 month

liver/biliary system
• mutants exhibit severe liver congestion

muscle
• cardiomyocytes are hypertrophic, showing an increase in cross-sectional area
• hearts show loss of desmosomes, however adherens junctions are normal
• hearts show reduced fractional shortening and ejection fraction
• 18 day old mutants exhibit extensive cardiomyocyte death, including apoptisis and necrosis
• mutants show rapid and progressive development of cardiomyopathy; hearts appear normal at P12 but by P18, show signs of fibrosis and heart enlargement by 1 month
• 18 day old mutants exhibit extensive cardiomyocyte death, including apoptisis and necrosis

cellular
• 18 day old mutants exhibit extensive cardiomyocyte death, including apoptisis and necrosis




Genotype
MGI:5502747
cn7
Allelic
Composition
Rhebtm1.1Otsu/Rhebtm1.1Otsu
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: 129 * C57BL/6J * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Rhebtm1.1Otsu mutation (0 available); any Rheb mutation (16 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype

Sarcomere maturation is attenuated in Rhebtm1.1Otsu/Rhebtm1.1Otsu Tg(Myh6-cre)2182Mds/0 hearts

mortality/aging
• mice die between P8 and P10

cardiovascular system
• reduced diastolic left ventricle posterior wall thickness
• larger end-diastolic and systolic dimensions
• reduced fractional shortening at P8
• however, cardiac function is normal until P5
• without the involvement of autophagy

muscle
• reduced fractional shortening at P8
• however, cardiac function is normal until P5
• without the involvement of autophagy
• sarcomere maturation is attenuated




Genotype
MGI:5502748
cn8
Allelic
Composition
Eif4ebp1tm1Nso/Eif4ebp1tm1Nso
Rhebtm1.1Otsu/Rhebtm1.1Otsu
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: 129 * C57BL/6J * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Eif4ebp1tm1Nso mutation (0 available); any Eif4ebp1 mutation (26 available)
Rhebtm1.1Otsu mutation (0 available); any Rheb mutation (16 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• improved survival compared to in Rhebtm1.1Otsu/Rhebtm1.1Otsu Tg(Myh6-cre)2182Mds mice

cardiovascular system
N
• mice exhibit improved cardiac function, hypertrophic growth and sarcomere maturation compared with Rhebtm1.1Otsu/Rhebtm1.1Otsu Tg(Myh6-cre)2182Mds mice




Genotype
MGI:3710677
cn9
Allelic
Composition
Rb1tm2Brn/Rb1tm2Brn
Tg(Myh6-cre)2182Mds/Tg(Myh6-cre)2182Mds
Genetic
Background
involves: 129 * FVB
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Rb1tm2Brn mutation (4 available); any Rb1 mutation (61 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
normal phenotype
• mutants are born with the expected Mendelian distribution and adult show no change in heart size, myocyte cell distribution, myocyte apoptosis, or mechanical function




Genotype
MGI:5314543
cn10
Allelic
Composition
Hprttm2(CAG-TBX2,-EGFP)Akis/Hprt+
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: 129 * FVB/N * NMRI
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Hprttm2(CAG-TBX2,-EGFP)Akis mutation (0 available); any Hprt mutation (1206 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• mice exhibit ectopic sub-endocardial mesenchyme in the atria and to a lesser extent in the ventricles unlike in control mice
• mice exhibit abnormal cardiac jelly and cushion formation in chamber myocardium compared with control mice




Genotype
MGI:3608600
cn11
Allelic
Composition
Srftm1Rjs/Srftm2.1Nor
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: 129P2/OlaHsd * 129S7/SvEvBrd * C57BL/6 * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Srftm1Rjs mutation (0 available); any Srf mutation (11 available)
Srftm2.1Nor mutation (0 available); any Srf mutation (11 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• no viable embryos are found beyond E12.5

cardiovascular system
• exhibit less constriction in the atrial ventricular canal at E10.5
• sarcomere organization is disrupted in hearts
• ventricular wall is hypoplastic with fewer trabeculae, ventricular wall compact layer is thinner, and cellular wall expansion is blocked
• intraventricular septation is less developed
• exhibit poorly developed intraventricular groove at E10.5
• dilated pericardial walls at E10.25
• seen at E10.25
• show severe pericardial effusion at E11.5
• lose rhythmic beating between E10.5 and E11.5

embryo
• some embryos arrest at the heart-looping stage (around E10.5-11.5)

muscle

homeostasis/metabolism
• show severe pericardial effusion at E11.5

cellular




Genotype
MGI:5477915
cn12
Allelic
Composition
Gja1tm10Kwi/Gja1tm10Kwi
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: 129P2/OlaHsd * C57BL/6 * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gja1tm10Kwi mutation (1 available); any Gja1 mutation (39 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• all mice die on the first day after birth

cardiovascular system
N
• mice exhibit normal heart beating frequency, PQ duration and QRS amplitude
• abnormal pouch formation at the subpulmonary outflow tract
• however, the outflow tract is open in neonates
• minor thickening and irregularities in the right ventricle
• 3-fold at E16.5

muscle
• minor thickening and irregularities in the right ventricle




Genotype
MGI:5317174
cn13
Allelic
Composition
Gjc1tm1.1(Gjd2)Kwi/Gjc1tm1.1(Gjd2)Kwi
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: 129P2/OlaHsd * C57BL/6 * FVB/N * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gjc1tm1.1(Gjd2)Kwi mutation (0 available); any Gjc1 mutation (21 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging

cardiovascular system
• fewer endocardial cells in the endocardial cushion tissue compared to in wild-type mice
• by E10.5 and E11.5
• mild by E10.5 and E11.5
• hearts contract weakly until E11.5
• at E10.5, the beating frequency of atria and ventricles is reduced compared to in wild-type mice
• at E10.5, mice exhibit impaired transmission atrial contraction impulses into the ventricles compared with wild-type mice
• at E10.5, most mice exhibit 2:1 or 3:1 AV blocks
• suggested by pericardial effusion and heart dilation at E10.5 and E11.5

embryo
• at E10.5 and E11.5
• at E10.5 and E11.5

growth/size/body
• at E10.5 and E11.5
• at E10.5 and E11.5

homeostasis/metabolism
• by E10.5 and E11.5

muscle
• hearts contract weakly until E11.5




Genotype
MGI:3687001
cn14
Allelic
Composition
Npr1tm1Kuhn/Npr1tm1Kuhn
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Npr1tm1Kuhn mutation (0 available); any Npr1 mutation (40 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• conditional mutants exhibit significantly increased ventricular cardiomyocyte diameters relative to homozygous Npr1tm1Kuhn mice, in the absence of interstitial fibrosis
• conditional mutants show a significant increase in the heart weight (HW) to body weight (BW) ratio relative to homozygous Npr1tm1Kuhn mice
• under baseline conditions, cardiac ventricles from conditional mutants show elevated expression levels of cardiac hypertrophy genes ANP, alpha-skeletal-actin, and beta-MHC (~4.9-fold, ~1.7-fold, and ~2-fold, respectively); the beta-MHC/alpha-MHC ratio is increased by ~2.7-fold
• in response to pressure overload induced by transverse aortic constriction (TAC), conditional mutants show an enhanced cardiac hypertrophic response, with a significantly lower SBP and induced pressure gradient, a greater LVW/BW index but a similar mortality rate relative to homozygous Npr1tm1Kuhn mice
• in response to pressure overload induced by TAC, conditional mutants show a slight increase in cardiac interstitial fibrosis relative to homozygous Npr1tm1Kuhn mice (24% vs 8%, respectively)
• conditional mutants show normal heart rates and left ventricular contractility relative to homozygous Npr1tm1Kuhn mice; in addition, chronotropic and inotropic responses to the beta-agonist dobutamine are preserved with no signs of cardiac dysfunction
• in contrast, baseline maximal relaxation rates and the lusitropic responses to low levels of beta-adrenergic stimulation are significantly reduced
• conditional mutants display slightly but significantly lower SBP relative to age- and sex-matched homozygous Npr1tm1Kuhn mice, by ~7-10 mmHg
• surprisingly, conditional mutants exhibit a mild but significant arterial hypotension by 7-10 mmHg
• in response to TAC-induced pressure load, conditional mutants display significant deterioration of cardiac function relative to TAC-operated homozygous Npr1tm1Kuhn mice
• at 10-14 days after TAC, conditional mutants show decreased LV contractility and relaxation as well as increased LV end-diastolic pressure and greater ratios of lung weight (LW) and right ventricular weight (RVW) to BW, indicating congestive heart failure without clinical signs of RV failure

muscle
• conditional mutants exhibit significantly increased ventricular cardiomyocyte diameters relative to homozygous Npr1tm1Kuhn mice, in the absence of interstitial fibrosis
• conditional mutants show normal heart rates and left ventricular contractility relative to homozygous Npr1tm1Kuhn mice; in addition, chronotropic and inotropic responses to the beta-agonist dobutamine are preserved with no signs of cardiac dysfunction
• in contrast, baseline maximal relaxation rates and the lusitropic responses to low levels of beta-adrenergic stimulation are significantly reduced

homeostasis/metabolism
• conditional mutants exhibit a >2-fold increase in plasma ANP levels relative to homozygous Npr1tm1Kuhn mice




Genotype
MGI:3721269
cn15
Allelic
Composition
Gab1tm1Nmoc/Gab1tm1Nmoc
Gab2tm1Thir/Gab2tm1Thir
Tg(Myh6-cre)2182Mds/?
Genetic
Background
involves: 129S1/Sv * 129X1/SvJ * C57BL/6J * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gab1tm1Nmoc mutation (1 available); any Gab1 mutation (56 available)
Gab2tm1Thir mutation (1 available); any Gab2 mutation (25 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• 70% of mice die between 3 and 72 weeks due to heart failure

cardiovascular system
• sarcomere length in the heart is reduced
• after 3 weeks, deposits of elastic fibers and collagen in the endocardium are observed
• at 10 weeks, the interventricular septal wall is thinned
• mice have a higher heart weight-to-body weight ratio when compared to control mice
• both the left and right ventricle are enlarged
• vessels in the left ventricle are abnormally dilated and are impaired in the recruitment of vascular smooth muscle cells
• mice that die of heart failure exhibit dilation of heart ventricles
• decrease in fractional shortening at 10 weeks of age
• left ventricle relaxation is impaired
• at 10 weeks, the left ventricle end-diastolic dimension is increased and accompanied by decreased fractional shortening
• 70% of mice die between 3 and 72 weeks due to heart failure

muscle
• decrease in fractional shortening at 10 weeks of age
• left ventricle relaxation is impaired




Genotype
MGI:3711507
cn16
Allelic
Composition
Cxadrtm1Mds/Cxadrtm1Mds
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: 129S1/Sv * 129X1/SvJ * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cxadrtm1Mds mutation (0 available); any Cxadr mutation (6 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
N
• many conditional null mice are born alive and survive to adulthood (20/102)

cardiovascular system
• sinuatrial valves show mild abnormalities in embryos, with only 1 embryo showing absence or attenuation comparable to constitutive null mice
• embryos show only mild or no abnormal ventricular thickening with little or no increase in proliferating cells




Genotype
MGI:3711512
cn17
Allelic
Composition
Cxadrtm1Mds/Cxadrtm1.1Mds
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: 129S1/Sv * 129X1/SvJ * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cxadrtm1.1Mds mutation (0 available); any Cxadr mutation (6 available)
Cxadrtm1Mds mutation (0 available); any Cxadr mutation (6 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
N
• many conditional null mice are born alive and survive to adulthood (20/102)

cardiovascular system
• sinuatrial valves show mild abnormalities in embryos, with only 1 embryo showing absence or attenuation comparable to constitutive null mice
• embryos show only mild or no abnormal ventricular thickening with little or no increase in proliferating cells




Genotype
MGI:3697609
cn18
Allelic
Composition
Acvr1tm1Vk/Acvr1tm1.1Vk
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: 129S1/Sv * 129X1/SvJ * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Acvr1tm1.1Vk mutation (0 available); any Acvr1 mutation (22 available)
Acvr1tm1Vk mutation (0 available); any Acvr1 mutation (22 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
N
• mutants are viable and fail to display any detectable altered cardiac phenotype




Genotype
MGI:3717096
cn19
Allelic
Composition
Shc1tm8Paw/Shc1tm9.1Paw
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: 129S1/Sv * 129X1/SvJ * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Shc1tm8Paw mutation (0 available); any Shc1 mutation (54 available)
Shc1tm9.1Paw mutation (0 available); any Shc1 mutation (54 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• between E12.5 and 13.5, 8/19 mutant embryos are dead, compared to all mutant embryos not expressing the cre-recombined allele; remaining embryos have regularly beating hearts and normal-appearing trabeculae




Genotype
MGI:4879021
cn20
Allelic
Composition
Stat3tm1Xyfu/Stat3+
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: 129S1/Sv * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Stat3tm1Xyfu mutation (1 available); any Stat3 mutation (43 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
immune system
• in the cardiac myocytes of LPS-treated mice
• LPS-treated mice exhibit increased TNF-alpha levels in cardiac myocytes compared with similarly treated wild-type mice although not as severely as in homozygous mice

cardiovascular system
• severe at 9 months




Genotype
MGI:4879018
cn21
Allelic
Composition
Stat3tm1Xyfu/Stat3tm1.1Xyfu
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: 129S1/Sv * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Stat3tm1.1Xyfu mutation (0 available); any Stat3 mutation (43 available)
Stat3tm1Xyfu mutation (1 available); any Stat3 mutation (43 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• after 6 months of age
• at 200 days, mice exhibit increased left ventricular chamber size compared with wild-type mice
• mild at 6 months
• severe at 9 months
• after 6 months of age
• doxorubicin-treated mice exhibit a greater decrease in cardiac systolic function compared with similarly treated wild-type mice
• LPS-treated mice exhibit increased myocyte apoptosis compared with similarly treated wild-type mice
• after 6 months of age

immune system
• in the cardiac myocytes of LPS-treated mice
• LPS-treated mice exhibit increased myocyte apoptosis and TNF-alpha levels in cardiac myocytes compared with similarly treated wild-type mice

homeostasis/metabolism
• after 6 months of age
• doxorubicin-treated mice exhibit a greater decrease in cardiac systolic function compared with similarly treated wild-type mice

respiratory system
• after 6 months of age

growth/size/body
• after 6 months of age

muscle
• LPS-treated mice exhibit increased myocyte apoptosis compared with similarly treated wild-type mice

cellular
• LPS-treated mice exhibit increased myocyte apoptosis compared with similarly treated wild-type mice




Genotype
MGI:3616710
cn22
Allelic
Composition
Mdm2tm1Glo/Mdm2tm2.1Glo
Tg(Myh6-cre)2182Mds/0
Trp53tm1Tyj/Trp53tm1Tyj
Genetic
Background
involves: 129S2/SvPas * 129S7/SvEvBrd * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mdm2tm1Glo mutation (0 available); any Mdm2 mutation (34 available)
Mdm2tm2.1Glo mutation (0 available); any Mdm2 mutation (34 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
Trp53tm1Tyj mutation (9 available); any Trp53 mutation (145 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
N
• exhibit rescue of the heart lethality seen in conditional Mdm2 mice and have the same life span as single homozygous Trp53 mice




Genotype
MGI:3807709
cn23
Allelic
Composition
Gja1tm8Kwi/Gja1+
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: 129S2/SvPas * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gja1tm8Kwi mutation (1 available); any Gja1 mutation (39 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• mutants are born live die during initial 6.5 months after birth
• 44% lethality occurs between E14.5 and 16.5

cardiovascular system
N
• mice do not differ significantly from wild-type in left ventricular (lv) muscle mass, lv end-diastolic volume and lv ejection fraction
• postnatally, mice do not differ significantly from wild-type in left ventricular (lv) muscle mass, lv end-diastolic volume and lv ejection fraction
• cardiac arrythmias are observed are observed after excorporation for ex vivo cardiac functional analyses including spontaneous and sustained ventricular tachycardias
• cardiac arrythmias are observed are observed after excorporation for ex vivo cardiac functional analyses including spontaneous and sustained ventricular tachycardias
• in vivo, spontaneous arrhythmic events such as ventricular extra systole (VES) are observed; frequency and severity of such events increase with by hypoxic stimulation
• surface and intercardiac ECGs ex vivo show a broadening of the QRS complex and decrease in the R wave in ventricle activity indicating disturbed impulse propagation
• decrease in the R wave
• isolated fetal cardiomyocytes show a 1.6-fold higher beating frequency than wild-type cells




Genotype
MGI:5297552
cn24
Allelic
Composition
Fstl1tm1.1Mvdh/Fstl1tm1.1Mvdh
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: 129S4/SvJae * C57BL/6 * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Fstl1tm1.1Mvdh mutation (0 available); any Fstl1 mutation (33 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• following pressure overload
• following pressure overload, the increase in myocardial capillary density is less than in wild-type mice
• following pressure overload, the increase in myocardial capillary density is less than in wild-type mice
• following pressure overload
• increased thickness following pressure overload
• following pressure overload
• following pressure overload
• increased left ventricular wall thickness following pressure overload
• following pressure overload
• following pressure overload
• increased left ventricular dimension and reduced fractional shortening following pressure overload
• following pressure overload, mice exhibit exacerbated cardiac myocyte hypertrophy and dysfunction compared with similarly treated wild-type mice

homeostasis/metabolism
• following pressure overload, mice exhibit exacerbated cardiac myocyte hypertrophy and dysfunction compared with similarly treated wild-type mice

muscle
• following pressure overload
• increased left ventricular dimension and reduced fractional shortening following pressure overload

respiratory system
• following pressure overload




Genotype
MGI:5304714
cn25
Allelic
Composition
Vhltm1Jae/Vhltm1Jae
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: 129S4/SvJae * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Myh6-cre)2182Mds mutation (3 available)
Vhltm1Jae mutation (2 available); any Vhl mutation (6 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• die between P16 and P18 due to sudden cardiac death
• mean survival is 9 weeks

cardiovascular system
• at 5 weeks and severe at 8 weeks
• at 8 weeks, mice exhibit reduced left ventricular wall thickness and increased left ventricular end-diastolic dimension compared with control mice
• at 5 weeks and severe at 8 weeks
• neonates exhibit decreased heart rate at 10 days after birth
• neonates exhibit frequent cardiac arrhythmia, consistent with sudden cardiac death
• neonates exhibit increased QRS at 10 days after birth
• exhibit increased QTc (c denotes correction for heart rate) and QTc dispersion at 10 days after birth
• severe at 8 weeks of age

muscle
• at 5 weeks and severe at 8 weeks
• severe at 8 weeks of age

cellular
• myocytes exhibit mitochondrial loss

Mouse Models of Human Disease
OMIM ID Ref(s)
Sudden Infant Death Syndrome 272120 J:193425




Genotype
MGI:3613580
cn26
Allelic
Composition
Mef2ctm1Eno/Mef2ctm1Jjs
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: 129S4/SvJaeSor * 129S6/SvEvTac * 129S7/SvEvBrd * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mef2ctm1Eno mutation (0 available); any Mef2c mutation (16 available)
Mef2ctm1Jjs mutation (1 available); any Mef2c mutation (16 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
normal phenotype
• no defects in heart or body weight parameters or in electrocardiogram analyses are seen in mice between 14 and 40 weeks of age
• suggests that this gene is not required for development or function of the heart after the looping morphogenesis stage




Genotype
MGI:5582621
cn27
Allelic
Composition
Grk5tm2Rjl/Grk5tm2Rjl
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: 129S4/SvJaeSor * C57BL/6J * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Grk5tm2Rjl mutation (1 available); any Grk5 mutation (26 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• 12 weeks after transaortic constriction, mice exhibit attenuated cardiac hypertrophy compared with wild-type mice
• 12 weeks after transaortic constriction, mice exhibit attenuated cardiac hypertrophy with no increase in left ventricular dilatation and no change in ejection fraction compared with wild-type mice

homeostasis/metabolism
• 12 weeks after transaortic constriction, mice exhibit attenuated cardiac hypertrophy with no increase in left ventricular dilatation and no change in ejection fraction compared with wild-type mice




Genotype
MGI:3616711
cn28
Allelic
Composition
Mdm4tm2Glo/Mdm4tm2.1Glo
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: 129S4/SvJaeSor * C57BL/6J * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mdm4tm2.1Glo mutation (0 available); any Mdm4 mutation (177 available)
Mdm4tm2Glo mutation (0 available); any Mdm4 mutation (177 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• some die within 1 year for unknown reasons, however do not observe any embryonic lethality

cardiovascular system
N
• do not observe any abnormalities in embryonic hearts




Genotype
MGI:3840255
cn29
Allelic
Composition
Ptpn11tm6Bgn/Ptpn11+
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: 129S6/SvEvTac * C57BL/6 * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ptpn11tm6Bgn mutation (2 available); any Ptpn11 mutation (25 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
N
• no gross cardiac defects are seen




Genotype
MGI:2687389
cn30
Allelic
Composition
Ednratm2Ywa/Ednratm2Ywa
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: 129S6/SvEvTac * C57BL/6J * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ednratm2Ywa mutation (1 available); any Ednra mutation (15 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
normal phenotype
• mutant mice are born at the expected Mendelian frequency and survive to adulthood in the absence of health problems
• at 6 weeks of age, mutants exhibit normal cardiac anatomy and weight, with no significant changes in histology of the right and left ventricular wall and interventricular septum relative to control mice
• when subjected to echocardiography, anesthetized 10-week-old male mutants exhibit normal cardiac contractility (as measured by peak velocity of left outflow tract (Vp), aortic velocity time integral, ejection time, and heart rate) relative to control mice
• surprisingly, in response to cardiac stress induced by 10-day infusion of angiotensin II or isoproterenol, male mutants display hypertrophic and contractile responses similar to those observed in control mice




Genotype
MGI:4946095
cn31
Allelic
Composition
Myh10tm7Rsad/Myh10tm7Rsad
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: 129S6/SvEvTac * C57BL/6J * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Myh10tm7Rsad mutation (1 available); any Myh10 mutation (53 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• widened and distorted in 4 of 5 mice
• at P0, 6 months, and 10 months with abnormal nuclei
• in 2 of 9 mice without double outlet heart right ventricle
• at 10 months, mice exhibit increased left ventricular internal diameter at the end of systole and decreased fractional shortening with abnormal intercalated disc compared with wild-type mice
• at 6 and 10 months

homeostasis/metabolism
• in the atrium of 2 in 8 mice at 10 months

immune system
• at 6 and 10 months

muscle
• widened and distorted in 4 of 5 mice
• at P0, 6 months, and 10 months with abnormal nuclei
• at 10 months, mice exhibit increased left ventricular internal diameter at the end of systole and decreased fractional shortening with abnormal intercalated disc compared with wild-type mice

cellular




Genotype
MGI:5304715
cn32
Allelic
Composition
Egln1tm1Kael/Egln1tm1Kael
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: 129S6/SvEvTac * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Egln1tm1Kael mutation (1 available); any Egln1 mutation (11 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• following thoracic aorta constriction, liver wet weight to dry weight is increased compared to in wild-type mice
• following thoracic aorta constriction, lung wet weight to dry weight is increased compared to in wild-type mice
• in aged mice
• following thoracic aorta constriction, mice exhibit myocyte dropout with replacement and interstitial fibrosis
• following thoracic aorta constriction
• following thoracic aorta constriction
• following thoracic aorta constriction, mice exhibit increased left ventricular end-diastolic dimension compared with control mice
• aged mice exhibit left ventricular posterior wall thickness compared with control mice
• following thoracic aorta constriction
• following thoracic aorta constriction or left anterior descending artery and in aged mice
• following thoracic aorta constriction, mice exhibit increased cardiac hypertrophy, reduced cardiac function (decreased fractional shortening and increased left ventricular end-diastolic dimension and left ventricular posterior wall thickness), increased heart weight, cardiac interstitial fibrosis, enlarge myocardial fiber, and congestion in the lung and liver compared with wild-type mice

muscle
• in aged mice
• following thoracic aorta constriction, mice exhibit myocyte dropout with replacement and interstitial fibrosis
• following thoracic aorta constriction or left anterior descending artery and in aged mice

homeostasis/metabolism
• following thoracic aorta constriction, mice exhibit increased cardiac hypertrophy, reduced cardiac function (decreased fractional shortening and increased left ventricular end-diastolic dimension and left ventricular posterior wall thickness), increased heart weight, cardiac interstitial fibrosis, enlarge myocardial fiber, and congestion in the lung and liver compared with wild-type mice

liver/biliary system
• following thoracic aorta constriction, liver wet weight to dry weight is increased compared to in wild-type mice

respiratory system
• following thoracic aorta constriction, lung wet weight to dry weight is increased compared to in wild-type mice

cellular
• myocytes exhibit mitochondrial loss




Genotype
MGI:3044594
cn33
Allelic
Composition
Edn1tm1Ywa/Edn1tm1Ywa
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: 129S6/SvEvTac * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Edn1tm1Ywa mutation (1 available); any Edn1 mutation (13 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• mice are resistant to hyperthyroid cardiac hypertrophy, showing a 57% reduction in cardiac hypertrophy in response to tri-iodothyronine (T3) relative to control mice (J:90390)
• left ventricular mass is increased by 3% in response to T3, compared to a 27% increase observed in similarly treated control mice (J:90390)
• the magnitude of Trypanosoma cruzi infection-associated cardiomyopathy is significantly less than in controls (J:96386)

homeostasis/metabolism
• mice are resistant to hyperthyroid cardiac hypertrophy, showing a 57% reduction in cardiac hypertrophy in response to tri-iodothyronine (T3) relative to control mice (J:90390)
• left ventricular mass is increased by 3% in response to T3, compared to a 27% increase observed in similarly treated control mice (J:90390)
• the magnitude of Trypanosoma cruzi infection-associated cardiomyopathy is significantly less than in controls (J:96386)




Genotype
MGI:3802690
cn34
Allelic
Composition
Gata4tm2(Gata4)Wtp/Gata4+
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: 129S6/SvEvTac * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gata4tm2(Gata4)Wtp mutation (1 available); any Gata4 mutation (15 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
phenotype not analyzed
• used for cell lineage tracing




Genotype
MGI:5304713
cn35
Allelic
Composition
Egln1tm1Kael/Egln1tm1Kael
Egln3tm1Vlcg/Egln3tm1Vlcg
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: 129S6/SvEvTac * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Egln1tm1Kael mutation (1 available); any Egln1 mutation (11 available)
Egln3tm1Vlcg mutation (0 available); any Egln3 mutation (10 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• mean survival is 29 weeks

cardiovascular system
• at 5 weeks and severe at 8 weeks
• at 8 weeks, mice exhibit reduced left ventricular wall thickness and increased left ventricular end-diastolic dimension compared with control mice
• at 5 weeks and severe at 8 weeks
• severe at 8 weeks of age

muscle
• at 5 weeks and severe at 8 weeks
• severe at 8 weeks of age

cellular
• myocytes exhibit mitochondrial loss




Genotype
MGI:5304716
cn36
Allelic
Composition
Gt(ROSA)26Sortm4(HIF2A*)Kael/Gt(ROSA)26Sor+
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: 129S6/SvEvTac * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gt(ROSA)26Sortm4(HIF2A*)Kael mutation (1 available); any Gt(ROSA)26Sor mutation (329 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• at 8 and 11 weeks, mice exhibit increased left ventricular end-diastolic dimension compared with control mice
• at 8 and 11 weeks
• at 8 and 11 weeks, mice exhibit reduced fractional shortening compared with control mice

muscle
• at 8 and 11 weeks, mice exhibit reduced fractional shortening compared with control mice




Genotype
MGI:3046803
cn37
Allelic
Composition
Tbx1tm1Bld/Tbx1tm3Bld
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: 129S7/SvEvBrd * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tbx1tm1Bld mutation (1 available); any Tbx1 mutation (15 available)
Tbx1tm3Bld mutation (1 available); any Tbx1 mutation (15 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• at E18.5 only those aortic arch abnormalities present in Tbx1tm1Bld heterozygotes are seen

Mouse Models of Human Disease
OMIM ID Ref(s)
DiGeorge Syndrome; DGS 188400 J:91013




Genotype
MGI:4837486
cn38
Allelic
Composition
Rassf1tm1.1Brd/Rassf1tm1.1Brd
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: 129S7/SvEvBrd * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Rassf1tm1.1Brd mutation (0 available); any Rassf1 mutation (12 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• following pressure overload hypertrophy, fibrosis and cardiomyocyte apoptosis are decreased and heart function is improved compared to wild-type controls
• in the absence of pressure overload, gross cardiac morphology is similar to controls

homeostasis/metabolism
• following pressure overload hypertrophy, fibrosis and cardiomyocyte apoptosis are decreased and heart function is improved compared to wild-type controls
• in the absence of pressure overload, gross cardiac morphology is similar to controls




Genotype
MGI:5490257
cn39
Allelic
Composition
Fkbp1atm1.1Shou/Fkbp1atm1Zuk
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: 129S7/SvEvBrd * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Fkbp1atm1.1Shou mutation (0 available); any Fkbp1a mutation (9 available)
Fkbp1atm1Zuk mutation (0 available); any Fkbp1a mutation (9 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
N
• cardiac development appears normal




Genotype
MGI:3616709
cn40
Allelic
Composition
Mdm2tm1Glo/Mdm2tm2.1Glo
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: 129S7/SvEvBrd * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mdm2tm1Glo mutation (0 available); any Mdm2 mutation (34 available)
Mdm2tm2.1Glo mutation (0 available); any Mdm2 mutation (34 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• all die by E13.5, with abnormalities evident at E9.5 but not E9

growth/size/body
• smaller than controls, probably due to lack of blood flow, however they develop to the correct developmental stage

cardiovascular system
• significant thinning of the myocardial layer in the ventricles
• heart mass is smaller in E9.5 embryos and by E13.5, most homozygotes lack any visible signs of a heart
• one of the E13.5 embryos that still has a heart shows abnormal trabeculation in the ventricle and abnormal ventricular wall structure
• hearts fail to function properly as indicated by blood leaking outside the heart and the backup of blood in the atrium
• decrease in blood flow that results in congestion and backup of blood in other organs
• exhibit blood leaking outside the heart

muscle

embryo
• smaller than controls, probably due to lack of blood flow, however they develop to the correct developmental stage

cellular




Genotype
MGI:3831703
cn41
Allelic
Composition
Hdac3tm1.1Eno/Hdac3tm1.1Eno
Tg(Myh6-cre)2182Mds/?
Genetic
Background
involves: 129S/SvEv * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Hdac3tm1.1Eno mutation (0 available); any Hdac3 mutation (14 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• significant lethality between 12 and 14 weeks of age
• 100% lethality by 16 weeks

cardiovascular system
N
• normal sinus rhythm
• 72% increase in heart weight/body weight ratio by 12 weeks of age
• signs of cardiac hypertrophy by 4 weeks of age
• hypertrophy exacerbated by 12 weeks of age
• both right and left atria are enlarged
• cardiomyocyte hypertrophy, particularly in the left ventricle free wall and the septum
• increased in both systole and diastole
• reduced fractional shortening of the left ventricle

cellular
• lack normal association with sarcomeres in cardiac muscle
• reduced density of cristae
• 25% reduction in complex I activity
• 39% reduction in NADH oxidase activity
• two fold increase in free radical production

homeostasis/metabolism
• accumulation of neutral lipids in ventricles but not atria
• fasting myocardial triglycerides are increased after 24 hours
• slightly reduced platelet counts

muscle
• reduced fractional shortening of the left ventricle




Genotype
MGI:3718385
cn42
Allelic
Composition
Hdac1tm1.1Eno/Hdac1tm1.1Eno
Tg(Myh6-cre)2182Mds/?
Genetic
Background
involves: 129S/SvEv * C57BL/6 * CD-1 * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Hdac1tm1.1Eno mutation (0 available); any Hdac1 mutation (21 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
normal phenotype
• mice are phenotypically normal and have no cardiac abnormalities




Genotype
MGI:3718388
cn43
Allelic
Composition
Hdac1tm1.1Eno/Hdac1tm1.1Eno
Hdac2tm1.1Eno/Hdac2tm1.1Eno
Tg(Myh6-cre)2182Mds/?
Genetic
Background
involves: 129S/SvEv * C57BL/6 * CD-1 * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Hdac1tm1.1Eno mutation (0 available); any Hdac1 mutation (21 available)
Hdac2tm1.1Eno mutation (0 available); any Hdac2 mutation (20 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging

cardiovascular system
• at P11, left and right ventricles are dilated
• at P10, mice display cardiac arrhythmias
• mice have a three-fold increase in apoptosis compared to wild-type and control mice

muscle
• mice have a three-fold increase in apoptosis compared to wild-type and control mice

cellular
• mice have a three-fold increase in apoptosis compared to wild-type and control mice




Genotype
MGI:3718387
cn44
Allelic
Composition
Hdac2tm1.1Eno/Hdac2tm1.1Eno
Tg(Myh6-cre)2182Mds/?
Genetic
Background
involves: 129S/SvEv * C57BL/6 * CD-1 * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Hdac2tm1.1Eno mutation (0 available); any Hdac2 mutation (20 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
normal phenotype
• mice are viable into adulthood and show no cardiac abnormalities




Genotype
MGI:3713114
cn45
Allelic
Composition
Atg5tm1Myok/Atg5tm1Myok
Tg(Myh6-cre)2182Mds/?
Genetic
Background
involves: 129S/SvEv * C57BL/6 * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Atg5tm1Myok mutation (2 available); any Atg5 mutation (18 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
normal phenotype
• mice are normal




Genotype
MGI:3778390
cn46
Allelic
Composition
Prkd1tm1Eno/Prkd1tm1Eno
Tg(Myh6-cre)2182Mds/?
Genetic
Background
involves: 129S/SvEv * C57BL/6 * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Prkd1tm1Eno mutation (1 available); any Prkd1 mutation (28 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• three weeks after surgical thoracic aortic constriction (TAC), mice have 50% less weight gain in the heart compared to wild-type mice
• mice only increase heart weight by 11% compared to 16% for wild-type mice after two weeks treatment with angiotensin II
• mice only increase heart weight by 21% compared to 37% increase in wild-type mice one week after isoproterenol treatment
• three weeks after TAC surgery, mice have minimal increase in left ventricle wall thickness unlike wild-type mice that have pronounced thickening of the left ventricle wall in response to TAC
• mice are resistant to dilation of left ventricle that occurs three weeks after TAC surgery in wild-type mice
• mice have much less fibrosis of the heart three weeks after TAC surgery compared to wild-type mice
• less fibrosis also occurs compared to wild-type mice in response to angiotensin II treatment
• mice do not display a decrease in fractional shortening three weeks after TAC surgery while wild-type mice have significant decreases
• mice are resistant to reductions in heart rate that occur three weeks after thoracic aortic constriction (TAC) surgery

muscle
• mice do not display a decrease in fractional shortening three weeks after TAC surgery while wild-type mice have significant decreases




Genotype
MGI:5502749
cn47
Allelic
Composition
Atg5tm1Myok/Atg5tm1Myok
Rhebtm1.1Otsu/Rhebtm1.1Otsu
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: 129S/SvEv * C57BL/6J * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Atg5tm1Myok mutation (2 available); any Atg5 mutation (18 available)
Rhebtm1.1Otsu mutation (0 available); any Rheb mutation (16 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging




Genotype
MGI:5544299
cn48
Allelic
Composition
Wwtr1tm1.1Eno/Wwtr1tm1.1Eno
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: 129S/SvEv * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Myh6-cre)2182Mds mutation (3 available)
Wwtr1tm1.1Eno mutation (0 available); any Wwtr1 mutation (11 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
N
• no obvious cardiac defects




Genotype
MGI:5544295
cn49
Allelic
Composition
Yap1tm1.1Eno/Yap1tm1.1Eno
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: 129S/SvEv * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Myh6-cre)2182Mds mutation (3 available)
Yap1tm1.1Eno mutation (0 available); any Yap1 mutation (25 available)
phenotype observed in females
phenotype observed in males
N normal phenotype

Lethal cardiomyopathy in Yap1tm1.1Eno/Yap1tm1.1Eno Tg(Myh6-cre)2182Mds/0 mice

mortality/aging
• die between 11 and 20 weeks of age from heart failure

cardiovascular system
• develops by 9 weeks of age
• mild dilation is seen at 6 weeks of age and becomes more pronounced at 12 weeks of age
• develops in older mice with dilated cardiomyopathy
• mild functional heart deficits by 6 weeks of age
• develops by 9 weeks of age and worsens with age
• mild at 6 weeks of age and becomes more pronounced at 12 weeks of age
• at 26 days after permanent ligation of the left anterior descending coronary artery at P2, mice display a gross deficiency of healthy myocardial tissue throughout the LV free wall and extensive fibrotic infarct zone unlike controls that have fully regenerated by this time

behavior/neurological
• become lethargic between 11 and 20 weeks of age

respiratory system
• develops between 11 and 20 weeks of age

homeostasis/metabolism
• at 26 days after permanent ligation of the left anterior descending coronary artery at P2, mice display a gross deficiency of healthy myocardial tissue throughout the LV free wall and extensive fibrotic infarct zone unlike controls that have fully regenerated by this time
• develops in older mice with dilated cardiomyopathy

muscle
• develops by 9 weeks of age and worsens with age
• mild at 6 weeks of age and becomes more pronounced at 12 weeks of age




Genotype
MGI:5544296
cn50
Allelic
Composition
Wwtr1tm1.1Eno/Wwtr1tm1.1Eno
Yap1tm1.1Eno/Yap1+
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: 129S/SvEv * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Myh6-cre)2182Mds mutation (3 available)
Wwtr1tm1.1Eno mutation (0 available); any Wwtr1 mutation (11 available)
Yap1tm1.1Eno mutation (0 available); any Yap1 mutation (25 available)
phenotype observed in females
phenotype observed in males
N normal phenotype

Cardiomyopathy in Wwtr1tm1.2Eno/Wwtr1tm1.2Eno Yap1tm1.1Eno/Yap1+ Tg(Myh6-cre)2182Mds/0 mice

mortality/aging
• complete lethality by 33 weeks of age from heart failure

cardiovascular system
• grossly dilated at 30 weeks of age but seen as early as 8 days of age

homeostasis/metabolism
• at 30 weeks of age

muscle
• grossly dilated at 30 weeks of age but seen as early as 8 days of age




Genotype
MGI:5544297
cn51
Allelic
Composition
Wwtr1tm1.1Eno/Wwtr1+
Yap1tm1.1Eno/Yap1tm1.1Eno
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: 129S/SvEv * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Myh6-cre)2182Mds mutation (3 available)
Wwtr1tm1.1Eno mutation (0 available); any Wwtr1 mutation (11 available)
Yap1tm1.1Eno mutation (0 available); any Yap1 mutation (25 available)
phenotype observed in females
phenotype observed in males
N normal phenotype

Cardiomyopathy in Wwtr1tm1.2Eno/Wwtr1+ Yap1tm1.1Eno/Yap1tm1.1Eno Tg(Myh6-cre)2182Mds/0 mice

mortality/aging
• die by 10 days of age
• lethality is accelerated compared to mutant mice wild type for Wwtr1

cardiovascular system
• about a 60% decrease in the number of cardiomyocytes

cellular

muscle




Genotype
MGI:5544298
cn52
Allelic
Composition
Wwtr1tm1.1Eno/Wwtr1tm1.1Eno
Yap1tm1.1Eno/Yap1tm1.1Eno
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: 129S/SvEv * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Myh6-cre)2182Mds mutation (3 available)
Wwtr1tm1.1Eno mutation (0 available); any Wwtr1 mutation (11 available)
Yap1tm1.1Eno mutation (0 available); any Yap1 mutation (25 available)
phenotype observed in females
phenotype observed in males
N normal phenotype

Wwtr1tm1.1Eno/Wwtr1tm1.1Eno Yap1tm1.1Eno/Yap1tm1.1Eno Tg(Myh6-cre)2182Mds/0 mice exhibit cardiac abnormalities and Wwtr1tm1.1Eno/Wwtr1+ Yap1tm1.1Eno/Yap1tm1.1Eno Tg(Myh6-cre)2182Mds/0 hearts show defects in proliferation and survival

mortality/aging

cardiovascular system
• severe cardiac defects




Genotype
MGI:5491820
cn53
Allelic
Composition
Rock2tm2Liao/Rock2tm2Liao
Tg(Myh6-cre)2182Mds/?
Genetic
Background
involves: 129/Sv * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Rock2tm2Liao mutation (0 available); any Rock2 mutation (30 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
N
• no anatomical or functional cardiac abnormalities before 25 weeks of age
• increase in systolic blood pressure in response to Ang II is similar to controls
• less fibrosis than controls from Ang II infusion
• less cardiac hypertrophy than controls from Ang II infusion
• less cardiac hypertrophy due to trans aortic constriction




Genotype
MGI:3689723
cn54
Allelic
Composition
Jarid2tm1Yskl/Jarid2tm1Yskl
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: 129/Sv * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Jarid2tm1Yskl mutation (0 available); any Jarid2 mutation (356 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
normal phenotype
• mice display no lethality up to 21 days after birth (P21); no detailed analyses have been done




Genotype
MGI:3802663
cn55
Allelic
Composition
Ppargc1atm1Dpk/Ppargc1atm1Dpk
Ppargc1btm1Dpk/Ppargc1btm1Dpk
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: 129X1/SvJ * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ppargc1atm1Dpk mutation (1 available); any Ppargc1a mutation (15 available)
Ppargc1btm1Dpk mutation (1 available); any Ppargc1b mutation (24 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• 67% of mice die within 24 hours of birth

cardiovascular system
• after birth, myocytes exhibit a reduction in mitochondrial number and size compared to in wild-type mice
• similar to that found in Ppargc1atm1Dpk Ppargc1btm1.1Dpk homozygotes




Genotype
MGI:3032514
cn56
Allelic
Composition
Rce1tm2Kim/Rce1tm2.1Kim
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Rce1tm2.1Kim mutation (0 available); any Rce1 mutation (7 available)
Rce1tm2Kim mutation (0 available); any Rce1 mutation (7 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• some mice died 3 to 5 months after birth
• 50% had died by 7 months of age
• 70% had died by 10 months of age

behavior/neurological
• mice were listless several weeks prior to death

cardiovascular system
• thin and dystrophic left ventricular musculature
• increased collagen between ventricular myoctes
• histological analysis revealed dilation of all four chambers

homeostasis/metabolism
• organized thrombi occasionally noted in left atria
• pleural and peritoneal fluid accumulation observed upon autopsy

muscle

integument
• observed several weeks prior to death




Genotype
MGI:4421539
cn57
Allelic
Composition
Abl1tm1Goff/Abl1tm1Goff
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Abl1tm1Goff mutation (1 available); any Abl1 mutation (71 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• most die shortly after birth

cardiovascular system
N
• compared to homozygous mutant mice not carrying the cre transgene, cardiomyocyte overproliferation and ventricular lumen size reduction are dramatically reduced
• not as thick as in homozygous mutant mice not carrying the cre transgene but slightly thicker than in heterozygous controls




Genotype
MGI:3045423
cn58
Allelic
Composition
Lpltm1Ijg/Lpltm1Ijg
Tg(Myh6-cre)2182Mds/?
Genetic
Background
involves: C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Lpltm1Ijg mutation (1 available); any Lpl mutation (19 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
homeostasis/metabolism
• basal glucose uptake by the heart increased 8 fold
• tendency for insulin stimulated glucose uptake by the heart to increase somewhat
• heart uptake of VLDL decreased by 72%
• heart content of fatty acids decreased
• postprandial levels tended to remain elevated
• plasma triglyceride levels significantly elevated in males because of increased VLDL triglycerides
• plasma triglyceride levels in females also tended to be elevated but not significantly
• heart content of triglycerides decreased
• postprandial levels tended to remain elevated

cardiovascular system
• basal glucose uptake by the heart increased 8 fold
• tendency for insulin stimulated glucose uptake by the heart to increase somewhat

muscle
• basal glucose uptake by the heart increased 8 fold
• tendency for insulin stimulated glucose uptake by the heart to increase somewhat

cellular
• basal glucose uptake by the heart increased 8 fold
• tendency for insulin stimulated glucose uptake by the heart to increase somewhat




Genotype
MGI:4830886
cn59
Allelic
Composition
Nox4tm1.1Jusa/Nox4tm1.1Jusa
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: C57BL/6 * FVB
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Nox4tm1.1Jusa mutation (0 available); any Nox4 mutation (24 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• reduced mortality in response to applied transverse aortic constriction induced pressure overload

cellular
• in response to pressure overload exhibit significantly decreased TUNEL positive cells compared to wild-type
• superoxide production, in the mitochondria in response to transverse aortic constriction induced pressure overload is abolished compared to wild-type
• Mitochondrial swelling, cytochrome c release, and a decrease in both mitochondrial DNA and aconitase activity in response to pressure overload are attenuated compared to wild-type
• decreased production of superoxide in left ventricular myocardial sections

cardiovascular system
• in response to pressure overload exhibit significantly attenuated cardiac hypertrophy
• in response to pressure overload exhibit significantly attenuated interstitial fibrosis
• decrease in echocardiographically evaluated LV ejection fraction (EF) and fractional shortening in response to transverse aortic constriction induced pressure overload
• decrease in echocardiographically evaluated LV ejection fraction (EF) and fractional shortening in response to transverse aortic constriction induced pressure overload
• in response to pressure overload exhibit significantly decreased TUNEL positive cells compared to wild-type
• better cardiac function compared to wild-type in response to transverse aortic constriction induced pressure overload

muscle
• decrease in echocardiographically evaluated LV ejection fraction (EF) and fractional shortening in response to transverse aortic constriction induced pressure overload
• decrease in echocardiographically evaluated LV ejection fraction (EF) and fractional shortening in response to transverse aortic constriction induced pressure overload
• in response to pressure overload exhibit significantly decreased TUNEL positive cells compared to wild-type

homeostasis/metabolism
• better cardiac function compared to wild-type in response to transverse aortic constriction induced pressure overload




Genotype
MGI:5526029
cn60
Allelic
Composition
Nr3c1tm1.1Jaci/Nr3c1tm1.1Jaci
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: C57BL/6 * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Nr3c1tm1.1Jaci mutation (0 available); any Nr3c1 mutation (12 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• increase morbidity and mortality by 5 months
• 7 months median survival age
• male mice have a median survival age of 8 months while this is only 6 months for female mice

respiratory system
• free erythrocytes in alveoli and hemosiderin-laden macrophages (heart failure cells) within alveolar spaces (at 6 months of age

growth/size/body
• in the majority of mice examined

immune system
• indicated by expression analysis

homeostasis/metabolism
• characterized by lamellation of fibrin and inflammatory cells, chronic inflammation, fibrosis, and neovascularization
• isolated cardiomyocytes fail to show a sustained increase in intracellular calcium following exposure to low concentrations of caffeine

muscle
• about a 140% increase in cardiomyocyte cross-sectional area at 3 months of age
• adrenalectomy at 2 months of age does not reduce the increase in cardiomyocyte size seen at 6 months of age
• variable decrease in ejection fraction and increase in the left ventricular end-systolic dimension at 3 months of age

cardiovascular system
N
• despite cardiac remodeling and reduced heart function, no electrocardiogram abnormalities are detected in mice at 3 - 6 months of age
• about a 140% increase in cardiomyocyte cross-sectional area at 3 months of age
• adrenalectomy at 2 months of age does not reduce the increase in cardiomyocyte size seen at 6 months of age
• in nearly all mice examined
• at 3 months of age
• at 3 months of age
• at 3 months
• associated with atrial thrombosis
• NORMAL: not detected in the ventricular wall at 3 months of age
• atrial thrombosis is consistent with atrial blood stasis
• associated with atrial thrombosis
• variable decrease in ejection fraction and increase in the left ventricular end-systolic dimension at 3 months of age
• no abnormality in 1 month-old mice but byat 3 months, but not at 1 month, mutant mice displays spontaneous LV left ventricular systolic dysfunction.
• isolated cardiomyocytes fail to show a sustained increase in intracellular calcium following exposure to low concentrations of caffeine
• indicated by expression analysis




Genotype
MGI:4836055
cn61
Allelic
Composition
Atp6ap2tm1.1Aich/Y
Tg(Myh6-cre)2182Mds/?
Genetic
Background
involves: C57BL/6 * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Atp6ap2tm1.1Aich mutation (0 available); any Atp6ap2 mutation (10 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• die within 3 weeks of birth
• born at expected ratios

cardiovascular system
• clusters of degenerating cardiomyocytes with extensive vacuolation in areas of replacement fibrosis
• myofibrils and mitochondria exclusively at the cell periphery of cardiomyocytes
• no gross cardiac anomalies at birth
• heart body ratio is significantly increased starting at day 14
• ventricular function severely impaired at 18 days of age
• fractional shortening in ventricles is 12.9% compared to 27.7% for controls at 18 days of age

muscle
• fractional shortening in ventricles is 12.9% compared to 27.7% for controls at 18 days of age




Genotype
MGI:5566535
cn62
Allelic
Composition
Rbm20tm2.1Hgra/Rbm20+
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: C57BL/6 * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Rbm20tm2.1Hgra mutation (0 available); any Rbm20 mutation (19 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• reduced stiffness under passive stress
• prolonged deceleration time of the E wave suggests a reduced diastolic left ventricular chamber stiffness

homeostasis/metabolism
• increased maximal running speed on a free-running wheel
• however, mean running distance is normal

muscle
• reduced stiffness under passive stress




Genotype
MGI:4421818
cn63
Allelic
Composition
Klf5tm2.1Rng/Klf5tm2.1Rng
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: C57BL/6 * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Klf5tm2.1Rng mutation (0 available); any Klf5 mutation (23 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype

Cardiomyocyte-specific deletion of Klf5 does not alter pressure overload-induced hypertrophy in Klf5tm2.1Rng/Klf5tm2.1Rng Tg(Myh6-cre)2182Mds/0 mice

cardiovascular system
N
• mice exhibit a normal response to low-intensity transverse aortic constriction




Genotype
MGI:5433520
cn64
Allelic
Composition
Hey2tm2.1Mtc/Hey2tm2.1Mtc
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: C57BL/6 * FVB/N * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Hey2tm2.1Mtc mutation (0 available); any Hey2 mutation (14 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• after pressure overload
• after pressure overload
• after pressure overload
• in cells from 12 week old mice
• however, treatment with FK506, an inhibitor of FKBP12.6, rescues defective cardiac function
• cardiomyocytes exhibit altered calcium cycling compared with control mice
• however, calcium sensitivity and stores are normal and treatment with FK506, an inhibitor of FKBP12.6, rescues defective peak calcium release
• after pressure overload, mice exhibit increased cardiac hypertrophy, decreased fractional shortening, fibrosis and heart failure compared with control mice
• after pressure overload
• however, mice do not exhibit cardiac failure under normal conditions

homeostasis/metabolism
• after pressure overload, mice exhibit increased cardiac hypertrophy, decreased fractional shortening, fibrosis and heart failure compared with control mice

muscle
• after pressure overload
• in cells from 12 week old mice
• however, treatment with FK506, an inhibitor of FKBP12.6, rescues defective cardiac function




Genotype
MGI:3525858
cn65
Allelic
Composition
Mapk14tm1.2Otsu/Mapk14tm1.2Otsu
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mapk14tm1.2Otsu mutation (1 available); any Mapk14 mutation (22 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• 30% of mutants died one week after TAC induced pressure overload compared to 8% in controls

cardiovascular system
• heart was larger than in controls after TAC induced pressure overload or adrenergic stimulation
• heart weight, left ventricle weight, and average ratio of left ventricle weight to tibial length or body weight were significantly greater than in controls after TAC induced pressure overload, however no difference in cardiomyocyte cell area was observed
• observed intermuscular and perivascular fibrosis in mutants compared to only slight perivascular fibrosis in controls after TAC induced pressure overload
• mice exhibit normal cardiac function and structure under baseline conditions, however cardiac contractility was significantly reduced after thoracic transverse aortic constriction (TAC) induced pressure overload and chronic adrenergic stimulation
• increased left ventricle end-diastolic and end-systolic diameters compared to controls after TAC induced pressure overload
• the number of apoptotic cardiac myocytes was 3.7 times that in controls after TAC induced pressure overload and was also increased in response to chronic adrenergic stimulation
• after TAC induced pressure overload, exhibit greater increases in mortality, heart size and weight, cardiac fibrosis, and cardiomyocyte apoptosis and decreased cardiac contractility compared to controls

muscle
• mice exhibit normal cardiac function and structure under baseline conditions, however cardiac contractility was significantly reduced after thoracic transverse aortic constriction (TAC) induced pressure overload and chronic adrenergic stimulation
• increased left ventricle end-diastolic and end-systolic diameters compared to controls after TAC induced pressure overload
• the number of apoptotic cardiac myocytes was 3.7 times that in controls after TAC induced pressure overload and was also increased in response to chronic adrenergic stimulation

homeostasis/metabolism
• after TAC induced pressure overload, exhibit greater increases in mortality, heart size and weight, cardiac fibrosis, and cardiomyocyte apoptosis and decreased cardiac contractility compared to controls

cellular
• the number of apoptotic cardiac myocytes was 3.7 times that in controls after TAC induced pressure overload and was also increased in response to chronic adrenergic stimulation




Genotype
MGI:3510547
cn66
Allelic
Composition
Raf1tm2Bacc/Raf1tm2Bacc
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Raf1tm2Bacc mutation (0 available); any Raf1 mutation (95 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• posterior wall thickness is significantly decreased
• the heart is enlarged; however, heart weight is not increased
• fractional shortening indicate that cardiac contractility is significantly decreased
• reduced maximal and minimal first derivatives of left ventricular pressure indicate that cardiac contractility and relaxation are significantly decreased
• increased apoptosis in the heart is seen from 3-5 weeks of age but not at 2 weeks or after 6 weeks of age

muscle
• fractional shortening indicate that cardiac contractility is significantly decreased
• reduced maximal and minimal first derivatives of left ventricular pressure indicate that cardiac contractility and relaxation are significantly decreased
• increased apoptosis in the heart is seen from 3-5 weeks of age but not at 2 weeks or after 6 weeks of age

cellular
• increased apoptosis in the heart is seen from 3-5 weeks of age but not at 2 weeks or after 6 weeks of age




Genotype
MGI:3510548
cn67
Allelic
Composition
Map3k5tm1Hijo/Map3k5tm1Hijo
Raf1tm2Bacc/Raf1tm2Bacc
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Map3k5tm1Hijo mutation (4 available); any Map3k5 mutation (34 available)
Raf1tm2Bacc mutation (0 available); any Raf1 mutation (95 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
N
• heart size, morphology and function are not significantly different from wild-type unlike Raf1tm2Bacc, Tg(Myhca-cre)2182Mds mutants




Genotype
MGI:5293311
cn68
Allelic
Composition
Capns1tm1.1Otsu/Capns1tm1.1Otsu
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: C57BL/6J * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Capns1tm1.1Otsu mutation (1 available); any Capns1 mutation (23 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype

Increased cardiac fibrosis in Capns1tm1.1Otsu/Capns1tm1.1Otsu Tg(Myh6-cre)2182Mds/0 mice after transverse aortic constriction

cardiovascular system
• in response to pressure overload or isoproterenol treatment, mice exhibit increased end-diastolic and end-systolic LV internal dimension compared with similarly treated wild-type mice
• 2 weeks after isoproterenol treatment
• in response to pressure overload
• in response to pressure overload or isoproterenol treatment
• mice exposed to pressure overload exhibit plasma membrane in heart sarcolemma compared with similarly treated wild-type mice
• heart sarcolemma exhibit defective membrane damage repair compared with similarly treated wild-type mice
• in response to pressure overload, mice exhibit congestive heart failure (increased end-diastolic and end-systolic LV internal dimension, decreased fractional shortening, and increased lung weight to body weight) compared with similarly treated wild-type mice
• mice treated with isoproterenol exhibit congestive heart failure (increased end-diastolic and end-systolic LV internal dimension, decreased fractional shortening, increased heart weight, and increased lung weight to body weight) compared with similarly treated wild-type mice
• in response to pressure overload or isoproterenol treatment

respiratory system
• in response to pressure overload or isoproterenol treatment

homeostasis/metabolism
• in response to pressure overload, mice exhibit congestive heart failure (increased end-diastolic and end-systolic LV internal dimension, decreased fractional shortening, and increased lung weight to body weight) compared with similarly treated wild-type mice
• mice treated with isoproterenol exhibit congestive heart failure (increased end-diastolic and end-systolic LV internal dimension, decreased fractional shortening, increased heart weight, and increased lung weight to body weight) compared with similarly treated wild-type mice

muscle
• in response to pressure overload or isoproterenol treatment




Genotype
MGI:5319195
cn69
Allelic
Composition
Fkbp1atm1.1Shou/Fkbp1atm1.1Shou
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Fkbp1atm1.1Shou mutation (0 available); any Fkbp1a mutation (9 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• shorter PP interval
• significant acceleration of the maximal phase 0 upstroke velocity in the left ventricle
• mean peak macroscopic voltage-gated Na+ current densities are increased more than 2 fold in ventricular cardiomyocytes
• acceleration of both the early and late component of voltage-gated Na+ current inactivation in ventricular cardiomyocytes




Genotype
MGI:3812382
cn70
Allelic
Composition
Cxadrtm1Mgot/Cxadrtm1Mgot
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cxadrtm1Mgot mutation (0 available); any Cxadr mutation (6 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• embryonic lethality occurs between E11.5 and E12.5




Genotype
MGI:3696414
cn71
Allelic
Composition
Map3k7tm1Mds/Map3k7tm1Mds
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Map3k7tm1Mds mutation (1 available); any Map3k7 mutation (38 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• lethality in midgestation, at the same time as seen in Map3k7Gt(Betageo-ALPP)1Byg homozygotes




Genotype
MGI:4461321
cn72
Allelic
Composition
Tg(CAG-Map3k7*K63W)1232Mds/0
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(CAG-Map3k7*K63W)1232Mds mutation (0 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• die by 11 days after birth

cardiovascular system
• disruption of the annulus fibrosus
• myocyte hypertrophy as indicated by increased myocyte diameter and induction of hypertrophic marker genes
• develop papillary muscle disorganization and fibrosis
• papillary muscle dysplasia
• heart-to-body weight ratio is normal at birth but is doubled by 7 days of age
• invariably develop ventricular wall thinning
• develop ventricular dilatation
• exhibit severely impaired ventricular filling and regurgitant flow into the left atrium
• PR interval shortening is evident at 1 day after birth and is further shortened by 4-8 days of age, indicating shortened AV conduction time
• ventricular QRS complex is initially normal, but all develop a slurred widened QRS morphology with an initial delta wave by 4-8 days of age
• glycogen storage cardiomyopathy

growth/size/body
• become growth retarded within 1 week after birth

homeostasis/metabolism
• develop left atrial thrombi
• cardiomyocyte glycogen content is elevated in hearts at 7 days of age

behavior/neurological
• exhibit decreased activity

muscle
• myocyte hypertrophy as indicated by increased myocyte diameter and induction of hypertrophic marker genes
• glycogen storage cardiomyopathy
• cardiomyocyte glycogen content is elevated in hearts at 7 days of age

vision/eye

Mouse Models of Human Disease
OMIM ID Ref(s)
Wolff-Parkinson-White Syndrome 194200 J:117108




Genotype
MGI:3655840
cn73
Allelic
Composition
Lpltm1Ijg/Lpltm1Ijg
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Lpltm1Ijg mutation (1 available); any Lpl mutation (19 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype

Increase in interstitial and perivascular collagen in Lpltm1Ijg/Lpltm1Ijg Tg(Myh6-cre)2182Mds/0 hearts

mortality/aging
• all mutants subjected to abdominal aortic constriction (AbAC) die within 2 days whereas controls survive

cardiovascular system
• hearts from non-stressed 4-5 month old males, but not females, show increased fibrosis as indicated by Trichrome staining; fibrosis is both interstitial and perivascular
• seen in non-stressed 4-5 month old males
• hearts from both 4-5 month old males and females exhibit higher rates of glycolysis and glucose oxidation
• palmitate oxidation rates in male and female hearts are reduced by 52 and 40%, respectively, indicating reduction in fatty acid utilization
• heart uptake of VLDL triglycerides is decreased by 49% and uptake of palmitate, a free fatty acid, is increased by 56% in females
• male, but not female, hearts show a reduction in cardiac power
• myocardial basal glucose uptake is increased 5.5-fold
• 6-month old mutants exhibit an increase in left ventricular systolic dimension and a decrease in fractional shortening under basal conditions
• mutants subjected to abdominal aortic constriction exhibit an increase in left ventricular end diastolic pressure that is not observed in banded controls
• mutants subjected to abdominal aortic constriction exhibit a greater increase in left ventricular systolic pressure than banded controls

homeostasis/metabolism
• 4 month old females show elevated plasma triglyceride levels, however plasma cholesterol, free fatty acid, and glucose levels are normal
• hearts from both 4-5 month old males and females exhibit higher rates of glycolysis and glucose oxidation

muscle
• myocardial basal glucose uptake is increased 5.5-fold
• 6-month old mutants exhibit an increase in left ventricular systolic dimension and a decrease in fractional shortening under basal conditions

cellular
• myocardial basal glucose uptake is increased 5.5-fold




Genotype
MGI:3639277
cn74
Allelic
Composition
Gata4tm1.1Wtp/Gata4tm1.1Wtp
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gata4tm1.1Wtp mutation (0 available); any Gata4 mutation (15 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging

cardiovascular system
• marked myocardial hyperplasia
• reduction in cardiomyocyte proliferation

muscle
• reduction in cardiomyocyte proliferation

cellular
• reduction in cardiomyocyte proliferation




Genotype
MGI:5314604
cn75
Allelic
Composition
Tg(CAG-cat,-TBX3)1Vmc/0
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(CAG-cat,-TBX3)1Vmc mutation (0 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• mice exhibit a subendocardial space near the atrioventricular cushions filled with mesenchymal cells unlike in control mice
• mice exhibit abnormal cardiac jelly and cushion formation in chamber myocardium compared with control mice
• mice exhibit hypoplastic chamber walls with reduced cellular proliferation in the ventricular walls compared to in control mice




Genotype
MGI:5543777
cn76
Allelic
Composition
Ehmt1tm2Yshk/Ehmt1tm2Yshk
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ehmt1tm2Yshk mutation (0 available); any Ehmt1 mutation (51 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
normal phenotype
• mice are born, develop and show no apparent abnormalities in appearance, cardiogenesis or fertility




Genotype
MGI:3051890
cn77
Allelic
Composition
Gja1tm1Gfi/Gja1tm1Gfi
Tg(Myh6-cre)2182Mds/0
Genetic
Background
Not Specified
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gja1tm1Gfi mutation (0 available); any Gja1 mutation (39 available)
Tg(Myh6-cre)2182Mds mutation (3 available)