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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Tg(Myh6-cre)2182Mds
transgene insertion 2182, Michael D Schneider
MGI:2386742
Summary 99 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
Gt(ROSA)26Sortm3(CAG-EYFP)Hze/Gt(ROSA)26Sor+
Hiratm1c(EUCOMM)Wtsi/Hiratm1d(EUCOMM)Wtsi
Tg(Myh6-cre)2182Mds/0
B6.Cg-Gt(ROSA)26Sortm3(CAG-EYFP)Hze Hiratm1c(EUCOMM)Wtsi/Hiratm1d(EUCOMM)Wtsi Tg(Myh6-cre)2182Mds MGI:5823159
cn3
Hand1tm1Eno/Hand1tm2Eno
Tg(Myh6-cre)2182Mds/0
either: (involves: 129S6/SvEvTac * Black Swiss) or (involves: 129S6/SvEvTac * C57BL/6) MGI:3514054
cn4
Rb1tm2Brn/Rb1tm2Brn
Rbl2tm1Tyj/Rbl2tm1Tyj
Tg(Myh6-cre)2182Mds/Tg(Myh6-cre)2182Mds
involves: 129 * 129S2/SvPas * FVB/N MGI:3710679
cn5
Cxadrtm1Know/Cxadrtm1Know
Tg(Myh6-cre)2182Mds/0
involves: 129 * Black Swiss * FVB/N MGI:3815088
cn6
Gata4tm1Grg/Gata4tm1.1Wtp
Tg(Myh6-cre)2182Mds/0
involves: 129 * C57BL/6 * FVB/N MGI:5427939
cn7
Juptm1.1Shou/Juptm1.1Shou
Tg(Myh6-cre)2182Mds/0
involves: 129 * C57BL/6J MGI:5296512
cn8
Rhebtm1.1Otsu/Rhebtm1.1Otsu
Tg(Myh6-cre)2182Mds/0
involves: 129 * C57BL/6J * FVB/N MGI:5502747
cn9
Eif4ebp1tm1Nso/Eif4ebp1tm1Nso
Rhebtm1.1Otsu/Rhebtm1.1Otsu
Tg(Myh6-cre)2182Mds/0
involves: 129 * C57BL/6J * FVB/N MGI:5502748
cn10
Rb1tm2Brn/Rb1tm2Brn
Tg(Myh6-cre)2182Mds/Tg(Myh6-cre)2182Mds
involves: 129 * FVB MGI:3710677
cn11
Hprttm2(CAG-TBX2,-EGFP)Akis/Hprt+
Tg(Myh6-cre)2182Mds/0
involves: 129 * FVB/N * NMRI MGI:5314543
cn12
Mdm4tm2Glo/Mdm4tm2.1Glo
Tg(Myh6-cre)2182Mds/0
Trp53tm1Tyj/Trp53tm1Brn
involves: 129P2/OlaHsd * 129S2/SvPas * 129S4/SvJaeSor * C57BL/6J * FVB/N MGI:5907135
cn13
Srftm1Rjs/Srftm2.1Nor
Tg(Myh6-cre)2182Mds/0
involves: 129P2/OlaHsd * 129S7/SvEvBrd * C57BL/6 * FVB/N MGI:3608600
cn14
Gja1tm10Kwi/Gja1tm10Kwi
Tg(Myh6-cre)2182Mds/0
involves: 129P2/OlaHsd * C57BL/6 * FVB/N MGI:5477915
cn15
Gjc1tm1.1(Gjd2)Kwi/Gjc1tm1.1(Gjd2)Kwi
Tg(Myh6-cre)2182Mds/0
involves: 129P2/OlaHsd * C57BL/6 * FVB/N * SJL MGI:5317174
cn16
Stat3tm1Vpo/Stat3+
Tg(Myh6-cre)2182Mds/0
involves: 129P2/OlaHsd * FVB/N MGI:5906909
cn17
Stat3tm1Vpo/Stat3tm1Vpo
Tg(Myh6-cre)2182Mds/0
involves: 129P2/OlaHsd * FVB/N MGI:5906908
cn18
Med1tm2Jkr/Med1tm2Jkr
Tg(Myh6-cre)2182Mds/0
involves: 129P2/OlaHsd * FVB/N MGI:5911326
cn19
Zfpm2tm1Sho/Zfpm2tm2Sho
Tg(Myh6-cre)2182Mds/0
involves: 129S1/Sv * 129S7/SvEvBrd MGI:3851401
cn20
Npr1tm1Kuhn/Npr1tm1Kuhn
Tg(Myh6-cre)2182Mds/0
involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * FVB/N MGI:3687001
cn21
Gab1tm1Nmoc/Gab1tm1Nmoc
Gab2tm1Thir/Gab2tm1Thir
Tg(Myh6-cre)2182Mds/?
involves: 129S1/Sv * 129X1/SvJ * C57BL/6J * FVB/N MGI:3721269
cn22
Cxadrtm1Mds/Cxadrtm1.1Mds
Tg(Myh6-cre)2182Mds/0
involves: 129S1/Sv * 129X1/SvJ * FVB/N MGI:3711512
cn23
Acvr1tm1Vk/Acvr1tm1.1Vk
Tg(Myh6-cre)2182Mds/0
involves: 129S1/Sv * 129X1/SvJ * FVB/N MGI:3697609
cn24
Shc1tm8Paw/Shc1tm9.1Paw
Tg(Myh6-cre)2182Mds/0
involves: 129S1/Sv * 129X1/SvJ * FVB/N MGI:3717096
cn25
Cxadrtm1Mds/Cxadrtm1Mds
Tg(Myh6-cre)2182Mds/0
involves: 129S1/Sv * 129X1/SvJ * FVB/N MGI:3711507
cn26
Stat3tm1Xyfu/Stat3+
Tg(Myh6-cre)2182Mds/0
involves: 129S1/Sv * FVB/N MGI:4879021
cn27
Stat3tm1Xyfu/Stat3tm1.1Xyfu
Tg(Myh6-cre)2182Mds/0
involves: 129S1/Sv * FVB/N MGI:4879018
cn28
Mdm4tm2Glo/Mdm4tm2.1Glo
Tg(Myh6-cre)2182Mds/0
Trp53tm1Tyj/Trp53+
involves: 129S2/SvPas * 129S4/SvJaeSor * C57BL/6J * FVB/N MGI:5907132
cn29
Mdm2tm1Glo/Mdm2tm2.1Glo
Tg(Myh6-cre)2182Mds/0
Trp53tm1Tyj/Trp53tm1Tyj
involves: 129S2/SvPas * 129S7/SvEvBrd * FVB/N MGI:3616710
cn30
Gja1tm8Kwi/Gja1+
Tg(Myh6-cre)2182Mds/0
involves: 129S2/SvPas * FVB/N MGI:3807709
cn31
Fstl1tm1.1Mvdh/Fstl1tm1.1Mvdh
Tg(Myh6-cre)2182Mds/0
involves: 129S4/SvJae * C57BL/6 * FVB/N MGI:5297552
cn32
Vhltm1Jae/Vhltm1Jae
Tg(Myh6-cre)2182Mds/0
involves: 129S4/SvJae * FVB/N MGI:5304714
cn33
Mef2ctm1Eno/Mef2ctm1Jjs
Tg(Myh6-cre)2182Mds/0
involves: 129S4/SvJaeSor * 129S6/SvEvTac * 129S7/SvEvBrd * FVB/N MGI:3613580
cn34
Grk5tm2Rjl/Grk5tm2Rjl
Tg(Myh6-cre)2182Mds/0
involves: 129S4/SvJaeSor * C57BL/6J * FVB/N MGI:5582621
cn35
Mdm4tm2Glo/Mdm4tm2.1Glo
Tg(Myh6-cre)2182Mds/0
involves: 129S4/SvJaeSor * C57BL/6J * FVB/N MGI:3616711
cn36
Hnrnputm1.1Tman/Hnrnputm1.1Tman
Tg(Myh6-cre)2182Mds/0
involves: 129S4/SvJaeSor * FVB/N MGI:5829559
cn37
Gata4tm1Sho/Gata4+
Tg(Myh6-cre)2182Mds/0
involves: 129S6/SvEvTac MGI:3851413
cn38
Gata4tm1Sho/Gata4tm1.1Wtp
Tg(Myh6-cre)2182Mds/0
involves: 129S6/SvEvTac * 129S7/SvEvBrd MGI:3851409
cn39
Esrrbtm1.1Nat/Esrrbtm1.1Nat
Tg(Myh6-cre)2182Mds/0
involves: 129S6/SvEvTac * C57BL/6 * C57BL/6N * FVB/N MGI:5905569
cn40
Ptpn11tm6Bgn/Ptpn11+
Tg(Myh6-cre)2182Mds/0
involves: 129S6/SvEvTac * C57BL/6 * FVB/N MGI:3840255
cn41
Ednratm2Ywa/Ednratm2Ywa
Tg(Myh6-cre)2182Mds/0
involves: 129S6/SvEvTac * C57BL/6J * FVB/N MGI:2687389
cn42
Myh10tm7Rsad/Myh10tm7Rsad
Tg(Myh6-cre)2182Mds/0
involves: 129S6/SvEvTac * C57BL/6J * FVB/N MGI:4946095
cn43
Edn1tm1Ywa/Edn1tm1Ywa
Tg(Myh6-cre)2182Mds/0
involves: 129S6/SvEvTac * FVB/N MGI:3044594
cn44
Egln1tm1Kael/Egln1tm1Kael
Tg(Myh6-cre)2182Mds/0
involves: 129S6/SvEvTac * FVB/N MGI:5304715
cn45
Egln1tm1Kael/Egln1tm1Kael
Egln3tm1Vlcg/Egln3tm1Vlcg
Tg(Myh6-cre)2182Mds/0
involves: 129S6/SvEvTac * FVB/N MGI:5304713
cn46
Ptger4tm1.1Matb/Ptger4tm1.1Matb
Tg(Myh6-cre)2182Mds/0
involves: 129S6/SvEvTac * FVB/N MGI:5906377
cn47
Gt(ROSA)26Sortm4(HIF2A*)Kael/Gt(ROSA)26Sor+
Tg(Myh6-cre)2182Mds/0
involves: 129S6/SvEvTac * FVB/N MGI:5304716
cn48
Gata4tm2(Gata4)Wtp/Gata4+
Tg(Myh6-cre)2182Mds/0
involves: 129S6/SvEvTac * FVB/N MGI:3802690
cn49
Tbx1tm1Bld/Tbx1tm3Bld
Tg(Myh6-cre)2182Mds/0
involves: 129S7/SvEvBrd * C57BL/6 MGI:3046803
cn50
Fkbp1atm1.1Shou/Fkbp1atm1Zuk
Tg(Myh6-cre)2182Mds/0
involves: 129S7/SvEvBrd * FVB/N MGI:5490257
cn51
Dicer1tm1Smr/Dicer1tm1Smr
Tg(Myh6-cre)2182Mds/0
involves: 129S7/SvEvBrd * FVB/N MGI:5906349
cn52
Rassf1tm1.1Brd/Rassf1tm1.1Brd
Tg(Myh6-cre)2182Mds/0
involves: 129S7/SvEvBrd * FVB/N MGI:4837486
cn53
Mdm2tm1Glo/Mdm2tm2.1Glo
Tg(Myh6-cre)2182Mds/0
involves: 129S7/SvEvBrd * FVB/N MGI:3616709
cn54
Hdac3tm1.1Eno/Hdac3tm1.1Eno
Tg(Myh6-cre)2182Mds/?
involves: 129S/SvEv * C57BL/6 MGI:3831703
cn55
Hdac2tm1.1Eno/Hdac2tm1.1Eno
Tg(Myh6-cre)2182Mds/?
involves: 129S/SvEv * C57BL/6 * CD-1 * FVB/N MGI:3718387
cn56
Hdac1tm1.1Eno/Hdac1tm1.1Eno
Tg(Myh6-cre)2182Mds/?
involves: 129S/SvEv * C57BL/6 * CD-1 * FVB/N MGI:3718385
cn57
Hdac1tm1.1Eno/Hdac1tm1.1Eno
Hdac2tm1.1Eno/Hdac2tm1.1Eno
Tg(Myh6-cre)2182Mds/?
involves: 129S/SvEv * C57BL/6 * CD-1 * FVB/N MGI:3718388
cn58
Prkd1tm1Eno/Prkd1tm1Eno
Tg(Myh6-cre)2182Mds/?
involves: 129S/SvEv * C57BL/6 * FVB/N MGI:3778390
cn59
Atg5tm1Myok/Atg5tm1Myok
Tg(Myh6-cre)2182Mds/?
involves: 129S/SvEv * C57BL/6 * FVB/N MGI:3713114
cn60
Atg5tm1Myok/Atg5tm1Myok
Rhebtm1.1Otsu/Rhebtm1.1Otsu
Tg(Myh6-cre)2182Mds/0
involves: 129S/SvEv * C57BL/6J * FVB/N MGI:5502749
cn61
Wwtr1tm1.1Eno/Wwtr1+
Yap1tm1.1Eno/Yap1tm1.1Eno
Tg(Myh6-cre)2182Mds/0
involves: 129S/SvEv * FVB/N MGI:5544297
cn62
Yap1tm1.1Eno/Yap1tm1.1Eno
Tg(Myh6-cre)2182Mds/0
involves: 129S/SvEv * FVB/N MGI:5544295
cn63
Wwtr1tm1.1Eno/Wwtr1tm1.1Eno
Yap1tm1.1Eno/Yap1+
Tg(Myh6-cre)2182Mds/0
involves: 129S/SvEv * FVB/N MGI:5544296
cn64
Wwtr1tm1.1Eno/Wwtr1tm1.1Eno
Yap1tm1.1Eno/Yap1tm1.1Eno
Tg(Myh6-cre)2182Mds/0
involves: 129S/SvEv * FVB/N MGI:5544298
cn65
Wwtr1tm1.1Eno/Wwtr1tm1.1Eno
Tg(Myh6-cre)2182Mds/0
involves: 129S/SvEv * FVB/N MGI:5544299
cn66
Rock2tm2Liao/Rock2tm2Liao
Tg(Myh6-cre)2182Mds/?
involves: 129/Sv * C57BL/6 MGI:5491820
cn67
Myocdtm1Msp/Myocdtm1Msp
Tg(Myh6-cre)2182Mds/0
involves: 129/Sv * FVB/N MGI:5907041
cn68
Jarid2tm1Yskl/Jarid2tm1Yskl
Tg(Myh6-cre)2182Mds/0
involves: 129/Sv * FVB/N MGI:3689723
cn69
Ppargc1atm1Dpk/Ppargc1atm1Dpk
Ppargc1btm1Dpk/Ppargc1btm1Dpk
Tg(Myh6-cre)2182Mds/0
involves: 129X1/SvJ * FVB/N MGI:3802663
cn70
Rce1tm2Kim/Rce1tm2.1Kim
Tg(Myh6-cre)2182Mds/0
involves: C57BL/6 MGI:3032514
cn71
Lpltm1Ijg/Lpltm1Ijg
Tg(Myh6-cre)2182Mds/?
involves: C57BL/6 MGI:3045423
cn72
Abl1tm1Goff/Abl1tm1Goff
Tg(Myh6-cre)2182Mds/0
involves: C57BL/6 MGI:4421539
cn73
Oma1tm1Tlan/Oma1tm1Tlan
Yme1l1tm1Tlan/Yme1l1tm1Tlan
Tg(Myh6-cre)2182Mds/0
involves: C57BL/6 * C57BL/6NCrl * FVB/N MGI:5805258
cn74
Yme1l1tm1Tlan/Yme1l1tm1Tlan
Tg(Myh6-cre)2182Mds/0
involves: C57BL/6 * C57BL/6NCrl * FVB/N MGI:5805256
cn75
Nox4tm1.1Jusa/Nox4tm1.1Jusa
Tg(Myh6-cre)2182Mds/0
involves: C57BL/6 * FVB MGI:4830886
cn76
Nr3c1tm1.1Jaci/Nr3c1tm1.1Jaci
Tg(Myh6-cre)2182Mds/0
involves: C57BL/6 * FVB/N MGI:5526029
cn77
Klf5tm2.1Rng/Klf5tm2.1Rng
Tg(Myh6-cre)2182Mds/0
involves: C57BL/6 * FVB/N MGI:4421818
cn78
Atp6ap2tm1.1Aich/Y
Tg(Myh6-cre)2182Mds/?
involves: C57BL/6 * FVB/N MGI:4836055
cn79
Rbm20tm2.1Hgra/Rbm20+
Tg(Myh6-cre)2182Mds/0
involves: C57BL/6 * FVB/N MGI:5566535
cn80
Hey2tm2.1Mtc/Hey2tm2.1Mtc
Tg(Myh6-cre)2182Mds/0
involves: C57BL/6 * FVB/N * SJL MGI:5433520
cn81
Map3k5tm1Hijo/Map3k5tm1Hijo
Raf1tm2Bacc/Raf1tm2Bacc
Tg(Myh6-cre)2182Mds/0
involves: C57BL/6J MGI:3510548
cn82
Mapk14tm1.2Otsu/Mapk14tm1.2Otsu
Tg(Myh6-cre)2182Mds/0
involves: C57BL/6J MGI:3525858
cn83
Raf1tm2Bacc/Raf1tm2Bacc
Tg(Myh6-cre)2182Mds/0
involves: C57BL/6J MGI:3510547
cn84
Cap2tm1c(EUCOMM)Wtsi/Cap2tm1c(EUCOMM)Wtsi
Tg(Myh6-cre)2182Mds/0
involves: C57BL/6J * C57BL/6N MGI:5806895
cn85
Capns1tm1.1Otsu/Capns1tm1.1Otsu
Tg(Myh6-cre)2182Mds/0
involves: C57BL/6J * FVB/N MGI:5293311
cn86
Snrktm2Rra/Snrk+
Tg(Myh6-cre)2182Mds/0
involves: C57BL/6J * FVB/N MGI:5792711
cn87
Snrktm2Rra/Snrktm2Rra
Tg(Myh6-cre)2182Mds/0
involves: C57BL/6J * FVB/N MGI:5792710
cn88
Lpltm1Ijg/Lpltm1Ijg
Tg(Myh6-cre)2182Mds/0
involves: FVB/N MGI:3655840
cn89
Gata4tm1.1Wtp/Gata4tm1.1Wtp
Tg(Myh6-cre)2182Mds/0
involves: FVB/N MGI:3639277
cn90
Tg(CAG-cat,-TBX3)1Vmc/0
Tg(Myh6-cre)2182Mds/0
involves: FVB/N MGI:5314604
cn91
Ehmt1tm2Yshk/Ehmt1tm2Yshk
Tg(Myh6-cre)2182Mds/0
involves: FVB/N MGI:5543777
cn92
Cxadrtm1Mgot/Cxadrtm1Mgot
Tg(Myh6-cre)2182Mds/0
involves: FVB/N MGI:3812382
cn93
Tg(CAG-Map3k7*K63W)1232Mds/0
Tg(Myh6-cre)2182Mds/0
involves: FVB/N MGI:4461321
cn94
Map3k7tm1Mds/Map3k7tm1Mds
Tg(Myh6-cre)2182Mds/0
involves: FVB/N MGI:3696414
cn95
Fkbp1atm1.1Shou/Fkbp1atm1.1Shou
Tg(Myh6-cre)2182Mds/0
involves: FVB/N MGI:5319195
cn96
Ttntm1Her/Ttntm1Her
Tg(Myh6-cre)2182Mds/0
Not Specified MGI:2651646
cn97
Gja1tm1Gfi/Gja1tm1Gfi
Tg(Myh6-cre)2182Mds/0
Not Specified MGI:3051890
cx98
Med13tm1Eno/Med13tm1Eno
Tg(Myh6-cre)2182Mds/0
involves: 129 * C57BL/6 * FVB/N MGI:5431523
tg99
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 (32 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:5823159
cn2
Allelic
Composition
Gt(ROSA)26Sortm3(CAG-EYFP)Hze/Gt(ROSA)26Sor+
Hiratm1c(EUCOMM)Wtsi/Hiratm1d(EUCOMM)Wtsi
Tg(Myh6-cre)2182Mds/0
Genetic
Background
B6.Cg-Gt(ROSA)26Sortm3(CAG-EYFP)Hze Hiratm1c(EUCOMM)Wtsi/Hiratm1d(EUCOMM)Wtsi Tg(Myh6-cre)2182Mds
Cell Lines EPD0181_1_A05
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gt(ROSA)26Sortm3(CAG-EYFP)Hze mutation (1 available); any Gt(ROSA)26Sor mutation (392 available)
Hiratm1c(EUCOMM)Wtsi mutation (1 available); any Hira mutation (58 available)
Hiratm1d(EUCOMM)Wtsi mutation (0 available); any Hira mutation (58 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• hearts show re-expression of fetal cardiac genes
• however, heart development proceeds normally
• hypertrophic cardiomyocytes are seen in the right ventricle free wall as early as 15 days after birth
• however, aberrant proliferation of or apoptosis of cardiomyocytes is not seen and there is no evidence of increased DNA damage
• lesions contain degenerating cardiomyocytes
• hearts at 6 weeks and 6 months of age exhibit white surface scars localized to the subepicardial region of the ventricular free walls; lesions contain degenerating cardiomyocytes and a large degree of collagen deposition indicating focal replacement fibrosis localized to the subepicardial myocardium
• mice show increased arterial elastance at 6 months of age, which is a measure of arterial load
• the slope of the end-diastolic pressure-volume relationship, which describes diastolic function, is elevated
• however, no effect is seen on the slope of end-systolic pressure-volume relationship, which describes the maximal developed ventricular pressure at any given ventricular volume
• mice show decreased stroke work at 6 months
• at 6 months of age
• at 6 months of age
• mice exhibit increased dp/dtmax-end-diastolic volume, the relationship between peak rate of pressure rise and EDV, which describes ventricular contractile performance
• mice show an increase in diastolic myocardial stiffness

cellular
• levels of manganese superoxide dismutase are reduced in scar-containing free-wall right ventricle, but not in non-scarred regions, suggesting impaired response to oxidative stress
• however, only very minor increases in reactive oxygen species are seen, indicating that cardiomyocyte degeneration most likely is not caused by increased oxidative stress

muscle
• hypertrophic cardiomyocytes are seen in the right ventricle free wall as early as 15 days after birth
• however, aberrant proliferation of or apoptosis of cardiomyocytes is not seen and there is no evidence of increased DNA damage
• lesions contain degenerating cardiomyocytes
• mice exhibit increased dp/dtmax-end-diastolic volume, the relationship between peak rate of pressure rise and EDV, which describes ventricular contractile performance
• cardiomyocyte sarcolemmal integrity is compromised in focal subepicardial areas between 15 and 25 days after birth




Genotype
MGI:3514054
cn3
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 (9 available)
Hand1tm2Eno mutation (0 available); any Hand1 mutation (9 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
cn4
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 (3 available); any Rb1 mutation (70 available)
Rbl2tm1Tyj mutation (1 available); any Rbl2 mutation (27 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
cn5
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
cn6
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 (23 available)
Gata4tm1Grg mutation (1 available); any Gata4 mutation (23 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
cn7
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 (147 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

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
arrhythmogenic right ventricular dysplasia 12 DOID:0110083 OMIM:611528
J:177567




Genotype
MGI:5502747
cn8
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 (20 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
cn9
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 (27 available)
Rhebtm1.1Otsu mutation (0 available); any Rheb mutation (20 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
cn10
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 (3 available); any Rb1 mutation (70 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
cn11
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 (1261 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:5907135
cn12
Allelic
Composition
Mdm4tm2Glo/Mdm4tm2.1Glo
Tg(Myh6-cre)2182Mds/0
Trp53tm1Tyj/Trp53tm1Brn
Genetic
Background
involves: 129P2/OlaHsd * 129S2/SvPas * 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 (181 available)
Mdm4tm2Glo mutation (0 available); any Mdm4 mutation (181 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
Trp53tm1Brn mutation (8 available); any Trp53 mutation (157 available)
Trp53tm1Tyj mutation (9 available); any Trp53 mutation (157 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• mice show an extended mean survival of 403 days compared to compound heterozygous Mdm4tm2Glo/Mdm4tm2.1Glo conditional mutants
• mice die due to tumors not heart failure

cardiovascular system
N
• mice do not exhibit signs of heart failure such as edema and poor breathing and exhibit rescue of the dilated cardiomyopathy

neoplasm




Genotype
MGI:3608600
cn13
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 (14 available)
Srftm2.1Nor mutation (0 available); any Srf mutation (14 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
cn14
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 (43 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
cn15
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 (23 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:5906909
cn16
Allelic
Composition
Stat3tm1Vpo/Stat3+
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: 129P2/OlaHsd * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Stat3tm1Vpo mutation (0 available); any Stat3 mutation (46 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• postpartum cardiomyopathy-related death is seen after 3-4 deliveries

cardiovascular system
• females develop postpartum cardiomyopathy
• after 4 pregnancies, the majority of females show signs of overt heart failure such as generalized edema and labored breathing

homeostasis/metabolism
• females exhibit generalized edema after 4 pregnancies

muscle
• females develop postpartum cardiomyopathy




Genotype
MGI:5906908
cn17
Allelic
Composition
Stat3tm1Vpo/Stat3tm1Vpo
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: 129P2/OlaHsd * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Stat3tm1Vpo mutation (0 available); any Stat3 mutation (46 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• about 2/3 of females die after the second pregnancy
• no females survive more than 5 pregnancies
• death always occurs within the first 3 weeks after delivery

cardiovascular system
• myocardial angiogenesis is impaired in postpartum females, with mice showing reduced left ventricle capillary density postpartum compared to controls
• increase in cardiomyocyte length in postpartum females
• extensive cardiac fibrosis in postpartum females
• 4-chamber dilatation in postpartum females
• depressed fractional shorting in postpartum females
• echocardiography indicates left ventricular dilatation and depressed fractional shorting in postpartum females
• cardiac apoptosis is increased in postpartum females
• all females develop postpartum cardiomyopathy
• treatment with tetrakis (4-benzoic acid) porphyrin (MnTBAP), a pharmacological suppressor of ROS, partially suppresses postpartum cardiomyopathy
• treatment with bromocriptine, a dopamine-D2-receptor agonist and inhibitor of prolactin secretion, prevents postpartum cardiomyopathy and mortality in females
• after 2 pregnancies, the majority of females show signs of overt heart failure such as generalized edema and labored breathing

cellular
• cardiac apoptosis is increased in postpartum females
• hearts of postpartum females exhibit increased oxidative stress

homeostasis/metabolism
• thrombi are often seen in the atria of postpartum females
• females exhibit generalized edema after 2 pregnancies

muscle
• increase in cardiomyocyte length in postpartum females
• depressed fractional shorting in postpartum females
• cardiac apoptosis is increased in postpartum females
• all females develop postpartum cardiomyopathy
• treatment with tetrakis (4-benzoic acid) porphyrin (MnTBAP), a pharmacological suppressor of ROS, partially suppresses postpartum cardiomyopathy
• treatment with bromocriptine, a dopamine-D2-receptor agonist and inhibitor of prolactin secretion, prevents postpartum cardiomyopathy and mortality in females

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
peripartum cardiomyopathy DOID:9997 J:141591




Genotype
MGI:5911326
cn18
Allelic
Composition
Med1tm2Jkr/Med1tm2Jkr
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: 129P2/OlaHsd * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Med1tm2Jkr mutation (1 available); any Med1 mutation (54 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• nearly 100% of mice die within 10 days post weaning

cardiovascular system
• lipid vacuoles of differing sizes are seen in cardiomyocytes
• cardiomyocytes show varying mitochondrial abnormalities, with some mitochondria containing lipid droplets and membranous swirls
• size of the heart increases by 22-29 days of age
• dilation of right and left ventricular chambers with thinning of the walls and myocardial cells
• dilated cardiomyopathy with atrial and ventricular dilatation
• at day 29, contractility of heart is diminished with a fractional shortening of 9.47% compared to 41.08% in controls and ejection fraction of 20.7% compared to 74.27% in controls
• echocardiography shows increased left ventricular end-diastolic internal dimension, decreased fractional shortening, and decreased ejection fraction
• increase in apoptosis in hearts, with an increase in the percentage of apoptotic cardiomyocytes

cellular
• increase in apoptosis in hearts, with an increase in the percentage of apoptotic cardiomyocytes
• mitochondrial DNA content is decreased in hearts

homeostasis/metabolism
• heart weight/body weight ratio is increased but dry heart weight is decreased, suggesting edematous heart
• lung weight/body weight ratio is increased most likely due to pulmonary edema

muscle
• lipid vacuoles of differing sizes are seen in cardiomyocytes
• cardiomyocytes show varying mitochondrial abnormalities, with some mitochondria containing lipid droplets and membranous swirls
• dilated cardiomyopathy with atrial and ventricular dilatation
• at day 29, contractility of heart is diminished with a fractional shortening of 9.47% compared to 41.08% in controls and ejection fraction of 20.7% compared to 74.27% in controls
• increase in apoptosis in hearts, with an increase in the percentage of apoptotic cardiomyocytes
• irregularities in Z band pattern

respiratory system
• lung weight/body weight ratio is increased most likely due to pulmonary edema




Genotype
MGI:3851401
cn19
Allelic
Composition
Zfpm2tm1Sho/Zfpm2tm2Sho
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: 129S1/Sv * 129S7/SvEvBrd
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Myh6-cre)2182Mds mutation (3 available)
Zfpm2tm1Sho mutation (2 available); any Zfpm2 mutation (27 available)
Zfpm2tm2Sho mutation (1 available); any Zfpm2 mutation (27 available)
phenotype observed in females
phenotype observed in males
N normal phenotype

Dilated cardiomyopathy in Zfpm2tm1Sho/Zfpm2tm2Sho Tg(Myh6-cre)2182Mds/0 mice

mortality/aging
• mice survive normally to weaning but die at 8-12 weeks

cardiovascular system
N
• at E14.5 no structural heart defects or coronary vascular plexus abnormalities are observed
• myocardium thickens normally and contains normal number of intramyocardial vessels
• decreased coronary vasculature is observed in adults
• density of PECAM positive vessels particularly capillaries are reduced in density; coronary arteriole density is also decreased
• significantly increased and replaces apoptotic tissues
• adult hearts are dilated
• ventricular dilation is observed
• decreased myocardial perfusion is observed in adults, resulting in tissue hypoxia
• fractional shortening is severely depressed in adults
• contraction is depressed in adults
• significantly increased in adult heart compared to controls

muscle
• fractional shortening is severely depressed in adults
• contraction is depressed in adults
• significantly increased in adult heart compared to controls

cellular
• significantly increased in adult heart compared to controls




Genotype
MGI:3687001
cn20
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 (45 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
cn21
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 (60 available)
Gab2tm1Thir mutation (1 available); any Gab2 mutation (26 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:3711512
cn22
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
cn23
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 (28 available)
Acvr1tm1Vk mutation (0 available); any Acvr1 mutation (28 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
cn24
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 (56 available)
Shc1tm9.1Paw mutation (0 available); any Shc1 mutation (56 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:3711507
cn25
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:4879021
cn26
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 (46 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
cn27
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 (46 available)
Stat3tm1Xyfu mutation (1 available); any Stat3 mutation (46 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
• severe at 9 months
• mild at 6 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:5907132
cn28
Allelic
Composition
Mdm4tm2Glo/Mdm4tm2.1Glo
Tg(Myh6-cre)2182Mds/0
Trp53tm1Tyj/Trp53+
Genetic
Background
involves: 129S2/SvPas * 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 (181 available)
Mdm4tm2Glo mutation (0 available); any Mdm4 mutation (181 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
Trp53tm1Tyj mutation (9 available); any Trp53 mutation (157 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• mice show an extended median survival of 274 days compared to compound heterozygous Mdm4tm2Glo/Mdm4tm2.1Glo conditional mutants




Genotype
MGI:3616710
cn29
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 (43 available)
Mdm2tm2.1Glo mutation (0 available); any Mdm2 mutation (43 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
Trp53tm1Tyj mutation (9 available); any Trp53 mutation (157 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
cn30
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 (43 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
cn31
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 (38 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
cn32
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 (10 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
DO ID OMIM ID(s) Ref(s)
sudden infant death syndrome DOID:9007 OMIM:272120
J:193425




Genotype
MGI:3613580
cn33
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
cn34
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 (28 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
cn35
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 (181 available)
Mdm4tm2Glo mutation (0 available); any Mdm4 mutation (181 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• some mice die within 1 year for unknown reasons, however do not observe any embryonic lethality (J:104114)
• median survival of 208 and 243 days for males and females, respectively (J:137114)
• mice die as a result of heart failure (J:137114)

cellular

cardiovascular system
N
• no abnormalities in embryonic hearts are seen
• hearts exhibit half the number of cardiomyocytes at 8 months of age
• marker analysis indicates cardiomyocyte hypertrophy
• ventricular walls are thinner and hypertrophic
• all 4 chambers are dilated and paler than control hearts
• severe dilated cardiomyopathy
• by 8-10 months of age, most mice have swollen bodies, have difficulty moving, and are out of breath

homeostasis/metabolism
• edema in the lung and/or abdomen

muscle
• hearts exhibit half the number of cardiomyocytes at 8 months of age
• marker analysis indicates cardiomyocyte hypertrophy
• severe dilated cardiomyopathy

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
dilated cardiomyopathy DOID:12930 OMIM:PS115200
J:137114




Genotype
MGI:5829559
cn36
Allelic
Composition
Hnrnputm1.1Tman/Hnrnputm1.1Tman
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: 129S4/SvJaeSor * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Hnrnputm1.1Tman mutation (0 available); any Hnrnpu mutation (27 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• although born at normal Mendelian ratios, all mice die abruptly from heart failure at exactly 10 days after birth

cardiovascular system
• mice develop severe, lethal dilated cardiomyopathy

muscle
• mice develop severe, lethal dilated cardiomyopathy




Genotype
MGI:3851413
cn37
Allelic
Composition
Gata4tm1Sho/Gata4+
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: 129S6/SvEvTac
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gata4tm1Sho mutation (0 available); any Gata4 mutation (23 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
N
• animals show normal development




Genotype
MGI:3851409
cn38
Allelic
Composition
Gata4tm1Sho/Gata4tm1.1Wtp
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: 129S6/SvEvTac * 129S7/SvEvBrd
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gata4tm1.1Wtp mutation (0 available); any Gata4 mutation (23 available)
Gata4tm1Sho mutation (0 available); any Gata4 mutation (23 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype

Reduced coronary microvasculature, decreased capillary density and increased fibrosis in Gata4tm1.1Wtp/Gata4tm1Sho Tg(Myh6-cre)2182Mds/0 hearts

cardiovascular system
• ventricular dilation is observed
• severely diminished systolic function at 8-14 weeks




Genotype
MGI:5905569
cn39
Allelic
Composition
Esrrbtm1.1Nat/Esrrbtm1.1Nat
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: 129S6/SvEvTac * C57BL/6 * C57BL/6N * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Esrrbtm1.1Nat mutation (1 available); any Esrrb mutation (233 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• poor survival rates after 9-10 months of age

cardiovascular system
• increase in heart weight to body weight and heart weight to tibial length ratios
• increase in fibrosis in the left ventricle
• mice develop adult-onset dilated cardiomyopathy associated with a decrease in anterior wall thickness, increased left ventricular end systolic diameter and left ventricular end diastolic diameter and a 60% decrease in fractional shortening
• decrease in cardiac function at 9 months of age but not at 4 and 6 months, with a 60% decrease in fractional shortening
• ventricular cardiomyocytes exhibit impaired contractility and show a decrease in both contractile distance (sarcomere shortening) and speed of contraction at 6 and 9 months of age
• ventricular cardiomyocytes exhibit impaired calcium handling and deregulation of the excitation-contraction coupling apparatus
• ventricular cardiomyocytes exhibit decreased calcium release and a trend towards decreased calcium uptake as early as 4 months of age
• by 6 months of age, calcium release and uptake by ventricular cardiomyocytes are increased and this persists at 9 months
• increase in calcium transients at 6 and 9 months of age
• expression analysis indicates a heart failure gene signature in hearts

muscle
• mice develop adult-onset dilated cardiomyopathy associated with a decrease in anterior wall thickness, increased left ventricular end systolic diameter and left ventricular end diastolic diameter and a 60% decrease in fractional shortening
• decrease in cardiac function at 9 months of age but not at 4 and 6 months, with a 60% decrease in fractional shortening
• ventricular cardiomyocytes exhibit impaired contractility and show a decrease in both contractile distance (sarcomere shortening) and speed of contraction at 6 and 9 months of age
• ventricular cardiomyocytes exhibit an increase in sarcomere length only after 9 months of age

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
dilated cardiomyopathy DOID:12930 OMIM:PS115200
J:241078




Genotype
MGI:3840255
cn40
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 (30 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
cn41
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 (20 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
cn42
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 (63 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:3044594
cn43
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 (20 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:5304715
cn44
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:5304713
cn45
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 (15 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

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
cardiomyopathy DOID:0050700 J:179490




Genotype
MGI:5906377
cn46
Allelic
Composition
Ptger4tm1.1Matb/Ptger4tm1.1Matb
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: 129S6/SvEvTac * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ptger4tm1.1Matb mutation (1 available); any Ptger4 mutation (16 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• length of myocytes from 31-33 week old males is increased, whereas width is normal
• heart weight to body weight ratio is increased in aged males
• males develop eccentric hypertrophy
• dilated left ventricle in males and a slight increase in left ventricular dimension in females
• males show reduced posterior wall thickness at diastole
• 39% increase in interstitial collagen fraction and greater collagen deposition in hearts
• older males show reduced ejection fraction and dilated left ventricle, indicating development of dilated cardiomyopathy
• females develop dilated cardiomyopathy at an older age and to a lesser extent than males
• ejection fraction is lower in 23-33 week old males; two groups are evident, those mice with ejection fraction greater than 70% and those with ejection fraction less than 70%
• 30-32 week old females show a modest, but significant, decline in ejection fraction
• a decline in ejection fraction begins around 16 weeks of age in males and after 24-28 weeks of age in females
• however, systolic blood pressure is normal in males and females at 28 weeks of age
• 28 week old male mice at with ejection fraction less than 70% exhibit increased left ventricular mass, increased left ventricular dimension at systole and left ventricular dimension at diastole, and reduced posterior wall thickness at diastole
• 31 week old females exhibit increased left ventricular dimension at systole
• patches of infiltration cells in hearts, however, the percentage of macrophages is not different from wild-type mice

immune system
• patches of infiltration cells in hearts, however, the percentage of macrophages is not different from wild-type mice

muscle
• older males show reduced ejection fraction and dilated left ventricle, indicating development of dilated cardiomyopathy
• females develop dilated cardiomyopathy at an older age and to a lesser extent than males
• ejection fraction is lower in 23-33 week old males; two groups are evident, those mice with ejection fraction greater than 70% and those with ejection fraction less than 70%
• 30-32 week old females show a modest, but significant, decline in ejection fraction
• a decline in ejection fraction begins around 16 weeks of age in males and after 24-28 weeks of age in females
• however, systolic blood pressure is normal in males and females at 28 weeks of age
• length of myocytes from 31-33 week old males is increased, whereas width is normal

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
dilated cardiomyopathy DOID:12930 OMIM:PS115200
J:158439




Genotype
MGI:5304716
cn47
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 (392 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
liver/biliary system

cardiovascular system
• at 8 and 11 weeks, mice exhibit increased left ventricular end-diastolic dimension compared with control mice
• at 8 and 11 weeks
• 8 week old mice show signs of dilated cardiomyopathy including left ventricle dilatation and decreased fractional shortening
• at 8 and 11 weeks, mice exhibit reduced fractional shortening compared with control mice

respiratory system

muscle
• 8 week old mice show signs of dilated cardiomyopathy including left ventricle dilatation and decreased fractional shortening
• at 8 and 11 weeks, mice exhibit reduced fractional shortening compared with control mice

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
cardiomyopathy DOID:0050700 J:179490




Genotype
MGI:3802690
cn48
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 (23 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:3046803
cn49
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 (17 available)
Tbx1tm3Bld mutation (1 available); any Tbx1 mutation (17 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
DO ID OMIM ID(s) Ref(s)
DiGeorge syndrome DOID:11198 OMIM:188400
J:91013




Genotype
MGI:5490257
cn50
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 (11 available)
Fkbp1atm1Zuk mutation (0 available); any Fkbp1a mutation (11 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:5906349
cn51
Allelic
Composition
Dicer1tm1Smr/Dicer1tm1Smr
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: 129S7/SvEvBrd * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Dicer1tm1Smr mutation (1 available); any Dicer1 mutation (59 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype

Dilated cardiomyopathy in Dicer1tm1Smr/Dicer1tm1Smr Tg(Myh6-cre)2182Mds/0 hearts

mortality/aging
• all mice die within 4 days after birth

behavior/neurological
• pups become fragile and exhibit decreased spontaneous activity preceding death

cardiovascular system
• neonatal cardiomyocytes show disarrayed myofibrils
• integrity of cardiomyocytes is impaired
• decrease in fractional shortening and an increase in the left ventricle posterior wall thickness at end diastole without change at end systole, indicating a loss of ventricle force generation
• hearts exhibit dysregulation of cardiac contractile protein expression
• however, sarcoplasmic reticulum calcium uptake rates are normal in hearts
• echocardiography indicates severe left ventricle dilation with a decrease in fractional shortening and an increase in the left ventricle posterior wall thickness at end diastole without change at end systole, indicating a loss of ventricle force generation
• hearts only beat about half of the rate
• increase in apoptosis in the left atrium of P2 hearts and in restricted areas of the ventricular apex and left ventricle wall, however no increase in apoptosis is seen before P0
• however, cardiomyocyte proliferation, cell size, and cell number are normal

cellular
• increase in apoptosis in the left atrium of P2 hearts and in restricted areas of the ventricular apex and left ventricle wall, however no increase in apoptosis is seen before P0
• however, cardiomyocyte proliferation, cell size, and cell number are normal

homeostasis/metabolism
• accumulation of blood clots and/or thrombin in the left atrium
• accumulation of blood clots and/or thrombin in the left ventricle and left atrium

muscle
• integrity of cardiomyocytes is impaired
• decrease in fractional shortening and an increase in the left ventricle posterior wall thickness at end diastole without change at end systole, indicating a loss of ventricle force generation
• hearts exhibit dysregulation of cardiac contractile protein expression
• however, sarcoplasmic reticulum calcium uptake rates are normal in hearts
• increase in apoptosis in the left atrium of P2 hearts and in restricted areas of the ventricular apex and left ventricle wall, however no increase in apoptosis is seen before P0
• however, cardiomyocyte proliferation, cell size, and cell number are normal
• less sarcomeres in heart ventricles and the sarcomeres that are present are disarrayed and shorter
• however, intercalated discs appear normal

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
dilated cardiomyopathy DOID:12930 OMIM:PS115200
J:131962




Genotype
MGI:4837486
cn52
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 (15 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:3616709
cn53
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 (43 available)
Mdm2tm2.1Glo mutation (0 available); any Mdm2 mutation (43 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
cn54
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 (15 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:3718387
cn55
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 (24 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:3718385
cn56
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 (25 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
cn57
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 (25 available)
Hdac2tm1.1Eno mutation (0 available); any Hdac2 mutation (24 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:3778390
cn58
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 (35 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:3713114
cn59
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 (19 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:5502749
cn60
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 (19 available)
Rhebtm1.1Otsu mutation (0 available); any Rheb mutation (20 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging




Genotype
MGI:5544297
cn61
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 (14 available)
Yap1tm1.1Eno mutation (0 available); any Yap1 mutation (27 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:5544295
cn62
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 (27 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
cn63
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 (14 available)
Yap1tm1.1Eno mutation (0 available); any Yap1 mutation (27 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:5544298
cn64
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 (14 available)
Yap1tm1.1Eno mutation (0 available); any Yap1 mutation (27 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:5544299
cn65
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 (14 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
N
• no obvious cardiac defects




Genotype
MGI:5491820
cn66
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 (35 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:5907041
cn67
Allelic
Composition
Myocdtm1Msp/Myocdtm1Msp
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: 129/Sv * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Myocdtm1Msp mutation (0 available); any Myocd mutation (33 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• about 20% of mice die before 3 weeks of age
• 90% of mice die by 10 months of age and no mice survive beyond 1 year of age

cardiovascular system
• both the left and right atrium are enlarged in 10 month old mice
• extensive loss of myofibrillar striations
• intercalated discs of hearts appear abnormal
• serpiginous intercalated discs with indistinct desmosomes and adherens junctions
• hearts show extensive loss of cardiomyocytes
• four-chamber enlargement in 10 month old mice
• both the left and right ventricles are enlarged in 10 month old mice
• 11.2% of the left ventricle myocardium shows fibrosis compared to 0.46% in controls
• mice develop lethal dilated cardiomyopathy
• systolic function is depressed
• mean ejection fraction is reduced (24.5% vs 57.8% in controls)
• echocardiography shows enlargement of the left atrium, left ventricle, right atrium, and right ventricle in 10 month old hearts

homeostasis/metabolism
• atrial thromboses are seen in 10 month old hearts
• atrial and ventricular thromboses are seen in 10 month old hearts

muscle
• extensive loss of myofibrillar striations
• intercalated discs of hearts appear abnormal
• serpiginous intercalated discs with indistinct desmosomes and adherens junctions