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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Ldlrtm1Her
targeted mutation 1, Joachim Herz
MGI:1857212
Summary 97 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
hm1
Ldlrtm1Her/Ldlrtm1Her B6.129S7-Ldlrtm1Her MGI:3772339
hm2
Ldlrtm1Her/Ldlrtm1Her B6.129S7-Ldlrtm1Her/J MGI:3783837
hm3
Ldlrtm1Her/Ldlrtm1Her involves: 129P2/OlaHsd * 129S7/SvEvBrd * C57BL/6 * DBA MGI:4358710
hm4
Ldlrtm1Her/Ldlrtm1Her involves: 129S6/SvEvTac * 129S7/SvEvBrd * C57BL/6 MGI:3719025
hm5
Ldlrtm1Her/Ldlrtm1Her involves: 129S7/SvEvBrd MGI:3691620
hm6
Ldlrtm1Her/Ldlrtm1Her involves: 129S7/SvEvBrd * C57BL/6 MGI:3611043
hm7
Ldlrtm1Her/Ldlrtm1Her involves: 129S7/SvEvBrd * C57BL/6J MGI:5661850
hm8
Ldlrtm1Her/Ldlrtm1Her involves: 129S7/SvEvBrd * C57BL/6 * SJL MGI:4367202
ht9
Ldlrtm1Her/Ldlr+ B6.129S7-Ldlrtm1Her MGI:4443062
ht10
Ldlrtm1Her/Ldlr+ involves: 129S7/SvEvBrd * C57BL/6 * DBA/2 MGI:3798392
cn11
Ldlrtm1Her/Ldlrtm1Her
Lrp1tm2Her/Lrp1tm2Her
Lyz2tm1(cre)Ifo/Lyz2+
involves: 129P2/OlaHsd * 129S7/SvEvBrd MGI:4943737
cn12
Apoetm1Lmh/Apoetm1Lmh
Ldlrtm1Her/Ldlrtm1Her
Lrp1tm2Her/Lrp1tm2Her
Lyz2tm1(cre)Ifo/Lyz2+
involves: 129P2/OlaHsd * 129S7/SvEvBrd MGI:4943551
cn13
Ldlrtm1Her/Ldlrtm1Her
Lrp1tm2Her/Lrp1tm2Her
Lyz2tm1(cre)Ifo/Lyz2+
involves: 129P2/OlaHsd * 129S7/SvEvBrd * C57BL/6J MGI:4943738
cn14
Ldlrtm1Her/Ldlrtm1Her
Lrp1tm2Her/Lrp1tm2Her
involves: 129S7/SvEvBrd MGI:4943555
cn15
Agtr1atm1Uky/Agtr1atm1Uky
Ldlrtm1Her/Ldlrtm1Her
Tg(Tek-cre)12Flv/0
involves: 129S7/SvEvBrd * C3H * C57BL/6 * C57BL/6N MGI:4946112
cn16
Agtr1atm1Uky/Agtr1atm1Uky
Ldlrtm1Her/Ldlrtm1Her
Tg(Tagln-cre)1Her/0
involves: 129S7/SvEvBrd * C57BL/6 * C57BL/6N * SJL MGI:4946109
cn17
Ldlrtm1Her/Ldlrtm1Her
Lrp1tm2Her/Lrp1tm2Her
Tg(Mx1-cre)29-4Her/0
involves: 129S7/SvEvBrd * C57BL/6J * SJL MGI:4943553
cn18
Apoetm1Lmh/Apoetm1Lmh
Ldlrtm1Her/Ldlrtm1Her
Lrp1tm2Her/Lrp1tm2Her
Tg(Mx1-cre)29-4Her/0
involves: 129S7/SvEvBrd * C57BL/6J * SJL MGI:3797226
cn19
Ldlrtm1Her/Ldlrtm1Her
Lrp1tm2Her/Lrp1tm2Her
Tg(Tagln-cre)1Her/0
involves: 129S7/SvEvBrd * C57BL/6J * SJL MGI:4943557
cn20
Ldlrtm1Her/Ldlrtm1Her
Lrp1tm2Her/Lrp1tm2Her
Tg(Mx1-cre)29-4Her/0
involves: 129S7/SvEvBrd * C57BL/6 * SJL MGI:3797225
cn21
Ldlrtm1Her/Ldlrtm1Her
Lrp1tm2Her/Lrp1tm2Her
Vldlrtm1Her/Vldlrtm1Her
Tg(Mx1-cre)29-4Her/0
involves: 129S7/SvEvBrd * C57BL/6 * SJL MGI:3797223
cn22
Ldlrtm1Her/Ldlrtm1Her
Nr1h3tm1.1Djm/Nr1h3tm1.1Djm
Tg(Alb-cre)21Mgn/0
involves: 129S/SvEv * 129S7/SvEvBrd * C57BL/6 * DBA MGI:5427004
cn23
Ldlrtm1Her/Ldlrtm1Her
Nr5a2tm2Sjns/Nr5a2tm2Sjns
Tg(Alb-cre)21Mgn/0
involves: 129/Sv * 129S7/SvEvBrd * C57BL/6 * C57BL/6J * DBA MGI:5607406
cx24
Apobec1tm1Ddsn/Apobec1tm1Ddsn
Ldlrtm1Her/Ldlrtm1Her
B6.129-Apobec1tm1Ddsn Ldlrtm1Her MGI:4367102
cx25
Apobec1tm1Ddsn/Apobec1tm1Ddsn
Fggtm1Fjc/Fggtm1Fjc
Ldlrtm1Her/Ldlrtm1Her
B6.129-Fggtm1Fjc Apobec1tm1Ddsn Ldlrtm1Her MGI:4367109
cx26
Agtr1atm1Unc/Agtr1atm1Unc
Ldlrtm1Her/Ldlrtm1Her
B6.129-Ldlrtm1Her Agtr1atm1Unc MGI:4946113
cx27
Apoa1tm1Unc/Apoa1tm1Unc
Ldlrtm1Her/Ldlrtm1Her
B6.129-Ldlrtm1Her Apoa1tm1Unc MGI:3783838
cx28
Gpr132tm1Witt/Gpr132tm1Witt
Ldlrtm1Her/Ldlrtm1Her
B6.129-Ldlrtm1Her Gpr132tm1Witt MGI:3639678
cx29
Ldlrtm1Her/Ldlrtm1Her
Tcra-Jtm1Tgi/Tcra-Jtm1Tgi
B6.129-Ldlrtm1Her Tcra-Jtm1Tgi MGI:4839086
cx30
Ifngtm1Ts/Ifngtm1Ts
Ldlrtm1Her/Ldlrtm1Her
B6.129S7-Ldlrtm1Her Ifngtm1Ts MGI:4867042
cx31
Cd1d1tm1Luc/Cd1d1tm1Luc
Ldlrtm1Her/Ldlrtm1Her
B6.129S-Cd1d1tm1Luc Ldlrtm1Her MGI:4821420
cx32
Ctsstm1Hap/Ctsstm1Hap
Ldlrtm1Her/Ldlrtm1Her
B6.129S-Ctsstm1Hap Ldlrtm1Her MGI:5008733
cx33
Del(11Cxcl16-Zmynd15)1Ifc/Del(11Cxcl16-Zmynd15)1Ifc
Ldlrtm1Her/Ldlrtm1Her
B6.129S-Ldlrtm1Her Del(11Cxcl16-Zmynd15)1Ifc MGI:3721540
cx34
Ldlrtm1Her/Ldlrtm1Her
Pfn1tm1Wit/Pfn1+
B6.129S-Ldlrtm1Her Pfn1tm1Wit MGI:4367099
cx35
Ldlrtm1Her/Ldlrtm1Her
Serpine1tm1Mlg/Serpine1tm1Mlg
B6.129S-Serpine1tm1Mlg Ldlrtm1Her MGI:3811068
cx36
Ldlrtm1Her/Ldlrtm1Her
Vwftm1Wgr/Vwftm1Wgr
B6.129S-Vwftm1Wgr Ldlrtm1Her MGI:4834596
cx37
Ldlrtm1Her/Ldlrtm1Her
Tlr2tm1Kir/Tlr2tm1Kir
B6.129-Tlr2tm1Kir Ldlrtm1Her MGI:5428446
cx38
Ldlrtm1Her/Ldlrtm1Her
Mobq5CAST/Ei/Mobq5CAST/Ei
B6.CAST-Mobq5CAST/Ei MGI:3711207
cx39
Ldlrtm1Her/Ldlrtm1Her
Mobq6CAST/Ei/Mobq6CAST/Ei
B6.CAST-Mobq6CAST/Ei MGI:3711214
cx40
Crptm1Hjf/Crptm1Hjf
Ldlrtm1Her/Ldlrtm1Her
B6.Cg-Crptm1Hjf Ldlrtm1Her MGI:4947401
cx41
Ins2Akita/Ins2Akita
Ldlrtm1Her/Ldlrtm1Her
B6.Cg-Ins2Akita Ldlrtm1Her MGI:5286555
cx42
Ldlrtm1Her/Ldlrtm1Her
Scd1ab-2J/Scd1ab-2J
B6.Cg-Ldlrtm1Her Scd1ab-2J MGI:3794980
cx43
Ldlrtm1Her/Ldlrtm1Her
Scd1ab-J/Scd1ab-J
B6.Cg-Ldlrtm1Her Scd1ab-J MGI:3794981
cx44
Ldlrtm1Her/Ldlrtm1Her
Tg(CMV-Serpine1)1Dgi/0
B6.Cg-Ldlrtm1Her Tg(CMV-Serpine1)1Dgi MGI:3810981
cx45
Ldlrtm1Her/Ldlr+
Lepob/Lepob
B6.Cg-Lepob Ldlrtm1Her MGI:3622658
cx46
Ldlrtm1Her/Ldlrtm1Her
Lepob/Lepob
B6.Cg-Lepob Ldlrtm1Her MGI:3622656
cx47
Apoetm1Unc/Apoetm1Unc
Ldlrtm1Her/Ldlrtm1Her
involves: 129P2/OlaHsd * 129S7/SvEvBrd MGI:4367224
cx48
Ldlrtm1Her/Ldlrtm1Her
Lipctm1Unc/Lipctm1Unc
involves: 129P2/OlaHsd * 129S7/SvEvBrd * C57BL/6 MGI:4367222
cx49
Ldlrtm1Her/Ldlrtm1Her
Mapkapk2tm1Mgl/Mapkapk2tm1Mgl
involves: 129P2/OlaHsd * 129S7/SvEvBrd * C57BL/6 MGI:4367112
cx50
Lcattm1Hgc/Lcattm1Hgc
Ldlrtm1Her/Ldlrtm1Her
involves: 129P2/OlaHsd * 129S7/SvEvBrd * C57BL/6 * DBA MGI:4358708
cx51
Apoetm1Unc/Apoetm1Unc
Ldlrtm1Her/Ldlrtm1Her
involves: 129P2/OlaHsd * 129S7/SvEvBrd * C57BL/6J MGI:3789482
cx52
Ddr1tm1Wfv/Ddr1tm1Wfv
Ldlrtm1Her/Ldlrtm1Her
involves: 129S1/Sv * 129S7/SvEvBrd * 129X1/SvJ MGI:4367117
cx53
Ldlrtm1Her/Ldlrtm1Her
Pcsk9tm1.2Prat/Pcsk9tm1.2Prat
involves: 129S1/Sv * 129S7/SvEvBrd * 129X1/SvJ MGI:4943544
cx54
Cxcl1tm1Wabo/Cxcl1tm1Wabo
Ldlrtm1Her/Ldlrtm1Her
involves: 129S1/Sv * 129S7/SvEvBrd * C57BL/6 MGI:3629493
cx55
Esr1tm1.1Mma/Esr1tm1.1Mma
Ldlrtm1Her/Ldlrtm1Her
involves: 129S2/SvPas * 129S7/SvEvBrd MGI:3833895
cx56
Itgb3tm1Hyn/Itgb3tm1Hyn
Ldlrtm1Her/Ldlrtm1Her
involves: 129S2/SvPas * 129S7/SvEvBrd * C57BL/6 MGI:4439281
cx57
Alox15tm1Fun/Alox15tm1Fun
Ldlrtm1Her/Ldlrtm1Her
involves: 129S2/SvPas * 129S7/SvEvBrd * C57BL/6 MGI:4367211
cx58
Apobec1tm1Chan/Apobec1tm1Chan
Ldlrtm1Her/Ldlrtm1Her
involves: 129S4/SvJae * 129S7/SvEvBrd MGI:3850551
cx59
Icam1tm1Jcgr/Icam1tm1Jcgr
Ldlrtm1Her/Ldlrtm1Her
involves: 129S4/SvJae * 129S7/SvEvBrd MGI:3620575
cx60
Ccl2tm1Rol/Ccl2tm1Rol
Ldlrtm1Her/Ldlrtm1Her
involves: 129S4/SvJae * 129S7/SvEvBrd * C57BL/6 MGI:4418562
cx61
Icam1tm1Jcgr/Icam1tm1Jcgr
Ldlrtm1Her/Ldlrtm1Her
Vcam1tm1Dmil/Vcam1tm1Dmil
involves: 129S4/SvJae * 129S7/SvEvBrd * C57BL/6 MGI:3620574
cx62
Ldlrtm1Her/Ldlrtm1Her
Vcam1tm1Dmil/Vcam1tm1Dmil
involves: 129S4/SvJae * 129S7/SvEvBrd * C57BL/6 MGI:3620572
cx63
Ldlrtm1Her/Ldlrtm1Her
Pparatm1Gonz/Pparatm1Gonz
involves: 129S4/SvJae * 129S7/SvEvBrd * C57BL/6 MGI:4367223
cx64
Apobec1tm1Ddsn/Apobec1tm1Ddsn
Ldlrtm1Her/Ldlrtm1Her
involves: 129S4/SvJae * 129S7/SvEvBrd * C57BL/6 MGI:4367101
cx65
Ldlrtm1Her/Ldlrtm1Her
Soat1tm1Far/Soat1tm1Far
involves: 129S4/SvJae * 129S7/SvEvBrd * C57BL/6J MGI:3761833
cx66
Ldlrtm1Her/Ldlrtm1Her
Scarb1tm1Dhu/Scarb1tm1Dhu
involves: 129S4/SvJae * BALB/c * C57BL/6J MGI:3623224
cx67
Angptl3tm1Lex/Angptl3tm1Lex
Ldlrtm1Her/Ldlrtm1Her
involves: 129S5/SvEvBrd * 129S7/SvEvBrd * C57BL/6 MGI:3849585
cx68
Ldlrtm1Her/Ldlrtm1Her
Tbx21tm1Glm/Tbx21tm1Glm
involves: 129S6/SvEvTac * 129S7/SvEvBrd * C57BL/6 MGI:3719026
cx69
Esr1tm1Arnal/Esr1tm1Arnal
Ldlrtm1Her/Ldlrtm1Her
involves: 129S7/SvEvBrd MGI:3833896
cx70
Ldlrtm1Her/Ldlrtm1Her
Olr1tm1Meht/Olr1tm1Meht
involves: 129S7/SvEvBrd * 129X1/SvJ * C57BL/6 MGI:3772340
cx71
Ldlrtm1Her/Ldlrtm1Her
Npc1m1N/Npc1m1N
involves: 129S7/SvEvBrd * BALB/c MGI:3785901
cx72
Ldlrtm1Her/Ldlrtm1Her
Npc1m1N/Npc1m1N
involves: 129S7/SvEvBrd * BALB/c * C57BL/6 MGI:4367225
cx73
Ldlrtm1Her/Ldlrtm1Her
Tg(APOC1)1Lmh/?
involves: 129S7/SvEvBrd * C57BL/6 MGI:3764683
cx74
Apobtm2Sgy/Apobtm2Sgy
Ldlrtm1Her/Ldlrtm1Her
involves: 129S7/SvEvBrd * C57BL/6 MGI:4367110
cx75
Apoetm1Lmh/Apoetm1Lmh
Ldlrtm1Her/Ldlrtm1Her
involves: 129S7/SvEvBrd * C57BL/6 MGI:3797227
cx76
Asgr2tm1Her/Asgr2tm1Her
Ldlrtm1Her/Ldlrtm1Her
involves: 129S7/SvEvBrd * C57BL/6 MGI:3511248
cx77
Soat1tm1Ishi/Soat1tm1Ishi
Ldlrtm1Her/Ldlrtm1Her
involves: 129S7/SvEvBrd * C57BL/6 MGI:3582203
cx78
Ldlrtm1Her/Ldlrtm1Her
Tg(APOM)NCchr/0
involves: 129S7/SvEvBrd * C57BL/6 * C57BL/6J * CBA MGI:3772662
cx79
Apobtm2Sgy/Apobtm2Sgy
Ldlrtm1Her/Ldlrtm1Her
Tg(Ins-Igf2)1Fbos/?
involves: 129S7/SvEvBrd * C57BL/6 * C57BL/6J * SJL MGI:5771865
cx80
Ldlrtm1Her/Ldlrtm1Her
Tg(Il1rn)1Dih/Tg(Il1rn)1Dih
involves: 129S7/SvEvBrd * C57BL/6 * CBA MGI:3574185
cx81
Ldlrtm1Her/Ldlrtm1Her
Tg(H2-K-AKR1B1)1Tj/0
involves: 129S7/SvEvBrd * C57BL/6 * DBA/2 MGI:3798391
cx82
Ldlrtm1Her/Ldlr+
Tg(H2-K-AKR1B1)1Tj/0
involves: 129S7/SvEvBrd * C57BL/6 * DBA/2 MGI:3798393
cx83
Ldlrtm1Her/Ldlrtm1Her
Nceh1tm1Ishi/Nceh1tm1Ishi
involves: 129S7/SvEvBrd * C57BL/6J MGI:4359432
cx84
Apobtm2Sgy/Apobtm2Sgy
Ldlrtm1Her/Ldlrtm1Her
involves: 129S7/SvEvBrd * C57BL/6J MGI:5771869
cx85
Ldlrtm1Her/Ldlrtm1Her
Lrpap1tm1Her/Lrpap1tm1Her
involves: 129S7/SvEvBrd * C57BL/6J MGI:3581865
cx86
Ldlrtm1Her/Ldlrtm1Her
Lipetm1Ishi/Lipetm1Ishi
involves: 129S7/SvEvBrd * C57BL/6J MGI:4359433
cx87
Ldlrtm1Her/Ldlrtm1Her
Lipetm1Ishi/Lipetm1Ishi
Nceh1tm1Ishi/Nceh1tm1Ishi
involves: 129S7/SvEvBrd * C57BL/6J MGI:4359435
cx88
Ldlrtm1Her/Ldlrtm1Her
Nr5a2tm3.1Sjns/Nr5a2tm3.1Sjns
involves: 129S7/SvEvBrd * C57BL/6J * C57BL/6N MGI:5607410
cx89
Ldlrtm1Her/Ldlrtm1Her
Tg(APOC3)3707Bres/?
involves: 129S7/SvEvBrd * C57BL/6J * CBA/J MGI:4367083
cx90
Ldlrtm1Her/Ldlrtm1Her
Tg(APOC3)3707Bres/?
Tg(Mt1-CETP)#Tall/?
involves: 129S7/SvEvBrd * C57BL/6J * CBA/J MGI:4941579
cx91
Apobtm2Sgy/Apobtm2Sgy
Ldlrtm1Her/Ldlrtm1Her
Tg(Ins-Igf2)1Fbos/?
involves: 129S7/SvEvBrd * C57BL/6 * SJL MGI:4367111
cx92
Ldlrtm1Her/Ldlrtm1Her
Tg(APPSWE)2576Kha/0
involves: 129S7/SvEvBrd * C57BL/6 * SJL MGI:4367205
cx93
Ldlrtm1Her/Ldlrtm1Her
Nr0b2tm1.1Mjev/Nr0b2tm1.1Mjev
involves: 129S/SvEvBrd * 129S4/SvJae * 129S7/SvEvBrd * C57BL/6 MGI:3847958
cx94
Cx3cl1tm1Sgs/Cx3cl1tm1Sgs
Ldlrtm1Her/Ldlrtm1Her
involves: B6.129S4-Cx3cl1tm1Sgs * B6.129S7-Ldlrtm1Her/J MGI:3527874
cx95
Cx3cl1tm1Sgs/Cx3cl1tm1Sgs
Ldlrtm1Her/Ldlr+
involves: B6.129S4-Cx3cl1tm1Sgs * B6.129S7-Ldlrtm1Her/J MGI:3527876
cx96
Ath37CAST/Ei/Ath37CAST/Ei
Ldlrtm1Her/Ldlrtm1Her
involves: CAST/Ei * C57BL/6J MGI:3639879
cx97
Ath38CAST/Ei/Ath38CAST/Ei
Ldlrtm1Her/Ldlrtm1Her
involves: CAST/Ei * C57BL/6J MGI:3639881


Genotype
MGI:3772339
hm1
Allelic
Composition
Ldlrtm1Her/Ldlrtm1Her
Genetic
Background
B6.129S7-Ldlrtm1Her
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
phenotype observed in females
phenotype observed in males
N normal phenotype

Adiposity in Ldlrtm1Her/Ldlrtm1Her and Scd1ab-2J/Scd1ab-2J Ldlrtm1Her/Ldlrtm1Her mice

homeostasis/metabolism
• streptozotocin (STZ) treatment leads to 3-fold higher level of blood glucose in mice fed a high fat diet (J:100916)
• these high glucose levels persist for at least 12 weeks after STZ treatment (J:100916)
• when fed a western style diet for 12 weeks (J:130278)
• when fed a western style diet for 12 weeks
• streptozotocin (STZ) induced diabetes leads to blood cholesterol levels that are twice those of non-diabetic mice 6 weeks post-induction and three times greater 8 weeks post- induction (J:100916)
• when fed a western style diet for 12 weeks, male mice exhibit a higher circulating cholesterol level than in Ldlrtm1Her Scd1ab-2J homozygotes (J:130278)
• when fed a high-cholesterol diet, NMR proton spectra of lipids indicates a level of photon intensity for HDL of 0.77 compared to 0.83 in wild-type mice
• mice develop severe cholesterolemia when receiving a high fat diet from 6 to 30 weeks (elevated levels are detected at 6, 15, and 30 weeks) (J:61287)
• seen at 3-4 months of age (J:72027)
• 10 fold increase in total cholesterol on a high fat diet compared to a 150% increase for controls (J:137264)
• 3 fold cholesterol elevation on a normal diet relative to controls (J:137264)
• slightly elevated at 3 and 8 months of age
• when fed a high cholesterol diet, NMR proton spectra of lipids indicates a level of photon intensity for LDL of 0.88 compared to 0.84 in wild-type mice (J:130794)
• compared to in Ldlrtm1Her Scd1ab-2J homozygotes when fed a western style diet for 12 weeks (J:130278)
• when fed a high cholesterol diet, NMR proton spectra of lipids indicates a level of photon intensity for VLDL of 0.92 compared to 0.88 in wild-type mice (J:130794)
• when fed a western style diet for 12 weeks
• livers exhibit slightly, but significantly, higher levels of cholesterol
• seen at 3-4 months of age (J:72027)
• when fed a western style diet for 12 weeks, female mice exhibit a higher circulating triglyceride level than in Ldlrtm1Her Scd1ab-2J homozygotes (J:130278)
• elevated on a high fat diet (J:137264)
• compared to in Ldlrtm1Her Scd1ab-2J homozygotes when fed a western style diet for 12 weeks
• when fed a western style diet for 12 weeks, mice exhibit a 5-fold higher hepatic triglyceride level than in Ldlrtm1Her Scd1ab-2J homozygotes
• increased amyloid beta 40 but not amyloid beta 42 on a high fat diet

adipose tissue
• increased when fed a western style diet for 12 weeks
• when fed a western style diet for 12 weeks

growth/size/body
• lower body weight on a low cholesterol diet than controls on any diet
• when fed a western style diet for 12 weeks

cardiovascular system
• high-fat diet leads to atherosclerosis (J:100916)
• STZ-induced diabetes leads to an almost 3-fold greater size in total lesion area in the aorta compared to non-diabetic Ldlr tm1Her homozyogotes (J:100916)
• 4 weeks on the Western diet mice have lesions of small fatty streaks on the aorta (J:110061)
• after 12 weeks on a high cholesterol diet, mice exhibit extensive intimal thickening and 60% to 80% of the aortic surface is sudanophilic unlike in wild-type mice (J:130794)
• after 12 weeks on a high cholesterol diet, mice exhibit endothelial disruption and an accumulation of macrophage and foam cells at the site of atherosclerotic plaques (J:130794)

immune system
• increased number of microglia in the hippocampus

liver/biliary system
• livers exhibit slightly, but significantly, higher levels of cholesterol
• when fed a western style diet for 12 weeks, mice exhibit a 5-fold higher hepatic triglyceride level than in Ldlrtm1Her Scd1ab-2J homozygotes
• when fed a western style diet for 12 weeks

behavior/neurological
• take longer to reach the target site in a Morris water maze when fed a high cholesterol diet
• deficient performance in a water radial arm maze regardless of the diet
• perform better than controls on a hanging bar test
• travel greater distances and spend more time in motion in an open field test

nervous system
• increased amyloid beta 40 but not amyloid beta 42 on a high fat diet
• increased number of microglia in the hippocampus
• increased numbers of reactive astrocytes
• further increase in reactive astrocytes on a high fat diet

hematopoietic system
• increased number of microglia in the hippocampus




Genotype
MGI:3783837
hm2
Allelic
Composition
Ldlrtm1Her/Ldlrtm1Her
Genetic
Background
B6.129S7-Ldlrtm1Her/J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
homeostasis/metabolism
N
• mice fed a Western diet exhibit normal HDL cholesterol
• when fed regular chow or a high fat diet for 16 weeks, serum triglyceride levels are decreased compared to similarly treated Apoa1tm1Unc Ldlrtm1Her homozygotes
• when fed a Western diet
• when fed a high fat diet, mice exhibit an increase in cholesterol content in the adrenal gland
• whether are fed a high fat diet or regular chow, plasma cholesterol levels are increased relative to similarly treated Apoa1tm1Unc Ldlrtm1Her homozygotes (J:85174)
• when fed a high fat diet, mice exhibit a greater increase in VLDL and LDL (6-fold) compared to Ldlrtm1Her homozygotes (3-fold) (J:85174)
• 15 fold higher on a normal diet than controls (J:104609)
• 40 fold higher than controls on a high fat diet (J:104609)
• when fed regular chow or a high fat diet for 16 weeks, serum HDL levels are increased compared to similarly treated Apoa1tm1Unc Ldlrtm1Her homozygotes
• when fed a high fat diet, mice exhibit a greater increase in VLDL and LDL (6-fold) compared to Ldlrtm1Her homozygotes (3-fold) (J:85174)
• when fed a Western diet compared to Ldlrtm1Her homozygotes fed regular chow (J:149005)
• when fed a high fat diet, mice exhibit a greater increase in VLDL and LDL (6-fold) compared to Ldlrtm1Her homozygotes (3-fold) (J:85174)
• when fed a Western diet (J:149005)
• when fed a high fat diet, mice exhibit a greater increase in liver cholesterol content (11-fold) compared to in similarly treated Apoa1tm1Unc Ldlrtm1Her homozygotes (2.5-fold)

liver/biliary system
• when fed a high fat diet, mice exhibit a greater increase in liver cholesterol content (11-fold) compared to in similarly treated Apoa1tm1Unc Ldlrtm1Her homozygotes (2.5-fold)
• when fed a high fat diet, mice exhibit larger diameter and more frequent lipid droplets than in Apoa1tm1Unc Ldlrtm1Her homozygotes
• LDL updake is decreased by about 2X

endocrine/exocrine glands
• when fed a high fat diet, mice exhibit an increase in cholesterol content in the adrenal gland

cardiovascular system
• after 8 or 20 weeks on a cholesterol diet aortic lesion size is increased compared to mice that are homozygous for both Ldlrtm1Her and Ifngtm1Ts

integument
• when fed a high fat diet
• when fed a high fat diet
• when fed a high fat diet




Genotype
MGI:4358710
hm3
Allelic
Composition
Ldlrtm1Her/Ldlrtm1Her
Genetic
Background
involves: 129P2/OlaHsd * 129S7/SvEvBrd * C57BL/6 * DBA
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
homeostasis/metabolism
• increase in APOB-100
• the HDL phospholipid fraction contains less 16:0 and 18:0 species and is enriched for 20:4 and 22:6 species compared to Apoetm1Unc single mutants
• plasma cholesterol is nearly equal distribution between the HDL and LDL fractions
• increase in the APOB lipoprotein cholesterol level compared to wild-type controls
• there is a 2.3 fold decrease in the ratio of saturated + monounsaturated/polyunsaturated cholesterol ester fatty acid species compared to Apoetm1Unc single mutants
• the ratio of saturated + monounsaturated/polyunsaturated cholesterol ester fatty acid species in the LDL fraction is significantly decreased compared to Apoetm1Unc single mutants




Genotype
MGI:3719025
hm4
Allelic
Composition
Ldlrtm1Her/Ldlrtm1Her
Genetic
Background
involves: 129S6/SvEvTac * 129S7/SvEvBrd * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
phenotype observed in females
phenotype observed in males
N normal phenotype

Phenotype of aortic arch atherosclerotic lesions in Tbx21tm1Glm/Tbx21tm1Glm Ldlrtm1Her/Ldlrtm1Her and Ldlrtm1Her/Ldlrtm1Her mice

cardiovascular system
• atherosclerosis in both aortic arch and descending aorta

homeostasis/metabolism




Genotype
MGI:3691620
hm5
Allelic
Composition
Ldlrtm1Her/Ldlrtm1Her
Genetic
Background
involves: 129S7/SvEvBrd
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
homeostasis/metabolism
• circulating VLDL/LDL cholesterol levels are increased compared to in Apobec1tm1Chan homozygotes and wild-type mice
• LDL clearance is slowed
• when fed a chow diet or Western-type diet for 2 and 4 weeks, mice exhibit increased serum cholesterol levels compared to in Apobec1tm1Chan homozygotes and wild-type mice (J:48202)
• plasma cholesterol level is 196mg/dl on a normal diet (J:84694)
• increased plasma cholesterol levels after 20 weeks on Western diet (J:228420)
• levels are higher than those exhibited by in transgenic Tg(Alb-MYLIP*)1Pton mice after 30 weeks on Western diet (J:228420)
• on a chow diet, plasma LDL levels were higher than both those of wild-type mice and Ldlrap1tm1Her homozygous mutant mice (J:84694)
• plasma LDL levels become further elevated on high cholesterol diets (J:84694)
• male mice have a marked increase in plasma LDL cholesterol compared to wild-type (J:114949)
• in wild-type mice parabiosed with Ldlr-null mice (resulting in shared circulation), plasma total cholesterol levels did not increase significantly over pre-surgery levels (J:114949)
• plasma levels are increased 2.5 fold relative to controls (J:169834)
• when fed a chow diet or a Western-type diet for 2 and 4 weeks, mice exhibit increased serum triglyceride levels compared to in Apobec1tm1Chan homozygotes and wild-type mice

cardiovascular system
• mice develop atherosclerotic lesions after 20 weeks on Western diet
• total lesion area is larger when compared to lesion area in transgenic Tg(Alb-MYLIP*)1Pton mice after 30 weeks on Western diet

Mouse Models of Human Disease
OMIM ID Ref(s)
Hypercholesterolemia, Familial 143890 J:84694




Genotype
MGI:3611043
hm6
Allelic
Composition
Ldlrtm1Her/Ldlrtm1Her
Genetic
Background
involves: 129S7/SvEvBrd * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
growth/size/body
• mice fed a diabetogenic high fat diet (35.5% carbohydrate and 36.6% fat) (DD diet) or a a diabetogenic high fat diet (35.5% carbohydrate and 36.6% fat) with 0.15% added cholesterol (DDC diet) exhibit weight gain leading to obesity; similar weight gain is seen regardless of diet

homeostasis/metabolism
• mothers on a high fat diet have reduced plasma concentrations of phenylalanine, lysine, valine, isoleucine, and leucine
• levels of other amino acids are normal
• levels in response to ACTH are significantly reduced
• similar levels of hypercholesterolemia are seen in mice fed the DD diet and those fed the DDC diet
• clearance of 125I-LDL from circulation is retarded, uptake of DiI-LDL by hepatocytes is decreased, and uptake of 3H-CE-LDL by the liver is lower compared to wild-type
• circulating fatty free acids are increased in mice fed the DD or the DDC diet, however levels are higher in the mice on the DDC diet
• similar levels of hypertriglyceridemia are seen in mice fed the DD diet and those fed the DDC diet
• ALT levels are increased in mice fed the DD diet and even more so in those fed the DDC diet
• increased fasting glucose levels after dexamethasone treatment
• after dexamethasone treatment
• glucose levels increase during a glucose tolerance test
• 30 minute insulin levels elevated
• less likely to become hypoglycemic during an insulin tolerance test
• hepatic cholesterol levels are increased only in the mice fed the DDC diet
• hepatic triglyceride levels are increased in mice fed the DD or the DDC diet, but higher in those on the DDC diet

nervous system
• on a Western diet, even small arterioles have associated microglia, sometimes within the basal lamina
• 2X as many arterioles have microglia
• reduced cell proliferation in the hippocampus
• reduced density of synaptophysin-immunoreactive presynaptic boutons in the CA1 of the hippocampus (J:120389)
• normal density of synaptophysin-immunoreactive presynaptic boutons in the dentate gyrus (J:120389)
• 32% of the cholesterol in the synaptic plasma membrane is in the exofacial leaflet as compared to 15% for controls
• cholesterol levels in the cytofacial leaflet are reduced
• cholesterol/phospholipid ratio in the synaptic plasma membrane is elevated
• fluidity of both the exofacial leaflet and the cytofacial leaflet of the synaptic plasma membrane are increased relative to controls

cardiovascular system
• wall thickness of brain arterioles having a lumen diameter of 15-40 um is greater than controls
• wall thickness increases on a Western diet
• wall thickness is directly related to the number of associated microglia
• thickened endothelial basal lamina
• reduced number of fenestrations
• narrowing of lumina

behavior/neurological
• mice on a Western diet fail to show improved performance over time in a Morris water maze test (J:116493)
• somewhat slower swimming speed in the acquisition phase of a Morris water maze test (J:120389)
• less time spent in the target quadrant during a probe test (J:120389)
• mice fed a Western diet and tested in a T-maze demonstrate reduced alternation returning more frequently to the blind arm of the maze (J:116493)

vision/eye
• thickened endothelial basal lamina
• reduced number of fenestrations
• narrowing of lumina
• decreased and irregular height
• basal membrane infoldings are less regular
• numerous vacuoles in cytoplasm
• thickened (up to 0.8um on a high fat diet)
• enhanced condensation of collagenous and elastic fibers
• laminations disrupted and large vacuoles become diffusely distributed

hematopoietic system
• on a Western diet, even small arterioles have associated microglia, sometimes within the basal lamina
• 2X as many arterioles have microglia

immune system
• on a Western diet, even small arterioles have associated microglia, sometimes within the basal lamina
• 2X as many arterioles have microglia
• inflammatory cell foci is seen to a greater extend in the livers of mice fed the DDC diet than the DD diet

pigmentation
• decreased and irregular height
• basal membrane infoldings are less regular
• numerous vacuoles in cytoplasm

cellular
• DD diet fed mice show some apoptotic cells in the liver while those on the DDC diet have a larger increase
• mice on the DDC diet exhibit an increase in hepatic oxidative stress
• poor survival of pups from mothers on a high fat diet
• intrauterine growth restriction of pups from mothers on a high fat diet
• also reduced birth weight persisting to at least 90 days of age
• offspring with lower gonadal fat pad to body weight ratio
• offspring with larger atherosclerotic lesions

liver/biliary system
• DD diet fed mice show some apoptotic cells in the liver while those on the DDC diet have a larger increase
• hepatic cholesterol levels are increased only in the mice fed the DDC diet
• hepatic triglyceride levels are increased in mice fed the DD or the DDC diet, but higher in those on the DDC diet
• inflammatory cell foci is seen to a greater extend in the livers of mice fed the DDC diet than the DD diet
• mice on the DD diet and on the DDC diet exhibit higher liver weights than regular chow-fed mice
• mice fed the DD diet exhibit diffuse macrovesicular steatosis with limited inflammation and fibrosis in the liver
• mice fed the DDC diet exhibit both macrovesicular and microvesicular steatosis in the liver
• intrasinusoidal and pericellular fibrosis is seen to a greater extend in the livers of mice fed the DDC diet than the DD diet




Genotype
MGI:5661850
hm7
Allelic
Composition
Ldlrtm1Her/Ldlrtm1Her
Genetic
Background
involves: 129S7/SvEvBrd * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• mice fed a cholate-free high-cholesterol diet (1%) for 6 months develop more extensive lesions throughout the aorta compared to controls which show smaller lesions that are almost exclusively in the aortic origin; the extent of atherosclerosis in the entire aorta correlates with the size of lesions in the aortic origin
• males show more aortic atherosclerosis than females, both in the arch and in the thoracic and abdominal aorta, whereas the average plasma cholesterol levels are similar in males and females
• when fed a high-cholesterol diet, mice develop more extensive atherosclerotic lesions than similarly fed controls, with males more affected than females

homeostasis/metabolism
• mice fed a high-fat diet (1% cholesterol) exhibit increased average total plasma cholesterol levels




Genotype
MGI:4367202
hm8
Allelic
Composition
Ldlrtm1Her/Ldlrtm1Her
Genetic
Background
involves: 129S7/SvEvBrd * C57BL/6 * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
homeostasis/metabolism
• total cholesterol greatly elevated




Genotype
MGI:4443062
ht9
Allelic
Composition
Ldlrtm1Her/Ldlr+
Genetic
Background
B6.129S7-Ldlrtm1Her
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
homeostasis/metabolism
• seen at 3-4 months of age
• seen at 3-4 months of age




Genotype
MGI:3798392
ht10
Allelic
Composition
Ldlrtm1Her/Ldlr+
Genetic
Background
involves: 129S7/SvEvBrd * C57BL/6 * DBA/2
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
homeostasis/metabolism
• streptozotocin (STZ) treatment leads to 3-fold higher level of blood glucose in mice fed a high fat diet
• these high glucose levels persist for at least 12 weeks after STZ treatment




Genotype
MGI:4943737
cn11
Allelic
Composition
Ldlrtm1Her/Ldlrtm1Her
Lrp1tm2Her/Lrp1tm2Her
Lyz2tm1(cre)Ifo/Lyz2+
Genetic
Background
involves: 129P2/OlaHsd * 129S7/SvEvBrd
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
Lrp1tm2Her mutation (1 available); any Lrp1 mutation (91 available)
Lyz2tm1(cre)Ifo mutation (7 available); any Lyz2 mutation (11 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
immune system
• impaired ability to internalize targets coated with LRP ligands
• however, phagocytosis of apoptotic cells in serum free conditions is similar to controls

hematopoietic system
• impaired ability to internalize targets coated with LRP ligands
• however, phagocytosis of apoptotic cells in serum free conditions is similar to controls




Genotype
MGI:4943551
cn12
Allelic
Composition
Apoetm1Lmh/Apoetm1Lmh
Ldlrtm1Her/Ldlrtm1Her
Lrp1tm2Her/Lrp1tm2Her
Lyz2tm1(cre)Ifo/Lyz2+
Genetic
Background
involves: 129P2/OlaHsd * 129S7/SvEvBrd
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Apoetm1Lmh mutation (0 available); any Apoe mutation (68 available)
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
Lrp1tm2Her mutation (1 available); any Lrp1 mutation (91 available)
Lyz2tm1(cre)Ifo mutation (7 available); any Lyz2 mutation (11 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• the total macrophage and collagen lesion contents are increased
• the percentage (normalized for lesion size) of collagen in lesions is increased; however, the percentages of macrophages and smooth muscle cells in the lesions are similar
• total atherosclerotic lesion area and the proportion of advanced lesions are significantly increased compared to mutant mice expressing Lrp1




Genotype
MGI:4943738
cn13
Allelic
Composition
Ldlrtm1Her/Ldlrtm1Her
Lrp1tm2Her/Lrp1tm2Her
Lyz2tm1(cre)Ifo/Lyz2+
Genetic
Background
involves: 129P2/OlaHsd * 129S7/SvEvBrd * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
Lrp1tm2Her mutation (1 available); any Lrp1 mutation (91 available)
Lyz2tm1(cre)Ifo mutation (7 available); any Lyz2 mutation (11 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
• significant decrease in the number of activated glial cells in ischemic tissue after transient middle cerebral artery occlusion
• significant decrease in the number of activated glial cells in ischemic tissue after transient middle cerebral artery occlusion
• significant reduction in the accumulation of nitrotyrosine in the ischemic tissue
• treatment with PLAT fails to restore nitrotyrosine accumulation unlike in mice null for Plat
• 24 hours after transient middle cerebral artery occlusion

homeostasis/metabolism
• significant decrease in the number of activated glial cells in ischemic tissue after transient middle cerebral artery occlusion
• significant reduction in the accumulation of nitrotyrosine in the ischemic tissue
• treatment with PLAT fails to restore nitrotyrosine accumulation unlike in mice null for Plat
• 24 hours after transient middle cerebral artery occlusion

immune system
• significant decrease in the number of activated glial cells in ischemic tissue after transient middle cerebral artery occlusion

hematopoietic system
• significant decrease in the number of activated glial cells in ischemic tissue after transient middle cerebral artery occlusion




Genotype
MGI:4943555
cn14
Allelic
Composition
Ldlrtm1Her/Ldlrtm1Her
Lrp1tm2Her/Lrp1tm2Her
Genetic
Background
involves: 129S7/SvEvBrd
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
Lrp1tm2Her mutation (1 available); any Lrp1 mutation (91 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
homeostasis/metabolism
• after adenoviral cre infection
• after adenoviral cre infection, concentrations of apoB48 are dramatically increased
• this increase is primarily responsible for the increase in plasma cholesterol and triglyceride levels
• slightly increased after adenoviral cre infection
• after adenoviral cre infection, the total cholesterol profile is dramatically changed
• increase in total plasma cholesterol levels after adenoviral cre infection
• large increase of plasma lipoproteins in the LDL size range after adenoviral cre infection
• large increase of plasma lipoproteins in the chylomicron remnant/VLDL size range after adenoviral cre infection




Genotype
MGI:4946112
cn15
Allelic
Composition
Agtr1atm1Uky/Agtr1atm1Uky
Ldlrtm1Her/Ldlrtm1Her
Tg(Tek-cre)12Flv/0
Genetic
Background
involves: 129S7/SvEvBrd * C3H * C57BL/6 * C57BL/6N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Agtr1atm1Uky mutation (1 available); any Agtr1a mutation (15 available)
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
Tg(Tek-cre)12Flv mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• angiotensin II-treated mice fed a high-fat diet exhibit decreased elastin breaks compared with similarly treated Ldlrtm1Her homozygotes
• angiotensin II-treated mice fed a high-fat diet exhibit decreased medial thickness compared with similarly treated Ldlrtm1Her homozygotes
• angiotensin II-treated mice fed a high-fat diet exhibit less ascending aorta lengthening compared with similarly treated Ldlrtm1Her homozygotes
• angiotensin II-treated mice fed a high-fat diet exhibit less ascending aorta ulcers compared with similarly treated Ldlrtm1Her homozygotes




Genotype
MGI:4946109
cn16
Allelic
Composition
Agtr1atm1Uky/Agtr1atm1Uky
Ldlrtm1Her/Ldlrtm1Her
Tg(Tagln-cre)1Her/0
Genetic
Background
involves: 129S7/SvEvBrd * C57BL/6 * C57BL/6N * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Agtr1atm1Uky mutation (1 available); any Agtr1a mutation (15 available)
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
Tg(Tagln-cre)1Her mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
N
• mice fed a high-fat diet exhibit the same amount of angiotensin-induced expansion of ascending aortas and aneurysms as in similarly treated Ldlrtm1Her homozygotes




Genotype
MGI:4943553
cn17
Allelic
Composition
Ldlrtm1Her/Ldlrtm1Her
Lrp1tm2Her/Lrp1tm2Her
Tg(Mx1-cre)29-4Her/0
Genetic
Background
involves: 129S7/SvEvBrd * C57BL/6J * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
Lrp1tm2Her mutation (1 available); any Lrp1 mutation (91 available)
Tg(Mx1-cre)29-4Her mutation (0 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
homeostasis/metabolism
• increase in total plasma cholesterol levels within 10 days of pIpC injection
• large increase of plasma lipoproteins in the LDL size range within 10 days of pIpC injection
• large increase of plasma lipoproteins in the chylomicron remnant/VLDL size range within 10 days of pIpC injection
• within 10 days of pIpC injection
• within 10 days of pIpC injection, concentrations of apoB48 are dramatically increased
• this increase is primarily responsible for the increase in plasma cholesterol and triglyceride levels




Genotype
MGI:3797226
cn18
Allelic
Composition
Apoetm1Lmh/Apoetm1Lmh
Ldlrtm1Her/Ldlrtm1Her
Lrp1tm2Her/Lrp1tm2Her
Tg(Mx1-cre)29-4Her/0
Genetic
Background
involves: 129S7/SvEvBrd * C57BL/6J * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Apoetm1Lmh mutation (0 available); any Apoe mutation (68 available)
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
Lrp1tm2Her mutation (1 available); any Lrp1 mutation (91 available)
Tg(Mx1-cre)29-4Her mutation (0 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
homeostasis/metabolism
• following pIpC treatment, plasma cholesterol level is lower compared to mutant mice wild-type for Lrp1
• following pIpC treatment, plasma LDL cholesterol level is lower compared to mutant mice wild-type for Lrp1
• following pIpC treatment, plasma VLDL cholesterol level is lower compared to mutant mice wild-type for Lrp1
• following pIpC treatment, plasma triglyceride level is lower compared to mutant mice wild-type for Lrp1
• increase in Factor VIII, von Willebrand factor, and tissue type plasminogen activator levels by 4 weeks after pIpC treatment (J:90547)
• mice show elevated FVIII (Factor 8) levels (J:117317)
• following pIpC treatment, plasma lipoprotein lipase level is higher compared to mutant mice wild-type for Lrp1
• however, no increase in lipoprotein lipase activity is detected

cardiovascular system
• increase in lesion size in pIpC treated mice compared to untreated controls
• however, no change in lesion composition is detected




Genotype
MGI:4943557
cn19
Allelic
Composition
Ldlrtm1Her/Ldlrtm1Her
Lrp1tm2Her/Lrp1tm2Her
Tg(Tagln-cre)1Her/0
Genetic
Background
involves: 129S7/SvEvBrd * C57BL/6J * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
Lrp1tm2Her mutation (1 available); any Lrp1 mutation (91 available)
Tg(Tagln-cre)1Her mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• progressive thickening of the aorta wall with age
• thickening is primarily caused by increased smooth muscle cell proliferation
• aortas are consistently distended and dilated
• pronounced atherosclerosis is seen in the aorta
• on a high cholesterol diet
• addition of Gleevec to the diet protects against atherosclerotic lesion formation
• almost complete occlusion of the mesenteric arteries
• increase in vascular smooth muscle cell proliferation

homeostasis/metabolism
N
• no changes in cholesterol or triglyceride levels are detected compared to mice homozygous for Ldlrtm1Her alone

muscle
• increase in vascular smooth muscle cell proliferation




Genotype
MGI:3797225
cn20
Allelic
Composition
Ldlrtm1Her/Ldlrtm1Her
Lrp1tm2Her/Lrp1tm2Her
Tg(Mx1-cre)29-4Her/0
Genetic
Background
involves: 129S7/SvEvBrd * C57BL/6 * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
Lrp1tm2Her mutation (1 available); any Lrp1 mutation (91 available)
Tg(Mx1-cre)29-4Her mutation (0 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
homeostasis/metabolism
• levels are significantly increased (>10-fold) compared to controls
• levels are significantly increased (>10-fold) compared to controls
• mice show significantly elevated FVIII (Factor 8) and VWF (von Willebrand factor) levels, by 4.2- and 3.3-fold respectively




Genotype
MGI:3797223
cn21
Allelic
Composition
Ldlrtm1Her/Ldlrtm1Her
Lrp1tm2Her/Lrp1tm2Her
Vldlrtm1Her/Vldlrtm1Her
Tg(Mx1-cre)29-4Her/0
Genetic
Background
involves: 129S7/SvEvBrd * C57BL/6 * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
Lrp1tm2Her mutation (1 available); any Lrp1 mutation (91 available)
Tg(Mx1-cre)29-4Her mutation (0 available)
Vldlrtm1Her mutation (1 available); any Vldlr mutation (31 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
homeostasis/metabolism
• mice show significantly elevated FVIII (Factor 8), similar to Ldlr/Lrp double mutants




Genotype
MGI:5427004
cn22
Allelic
Composition
Ldlrtm1Her/Ldlrtm1Her
Nr1h3tm1.1Djm/Nr1h3tm1.1Djm
Tg(Alb-cre)21Mgn/0
Genetic
Background
involves: 129S/SvEv * 129S7/SvEvBrd * C57BL/6 * DBA
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
Nr1h3tm1.1Djm mutation (0 available); any Nr1h3 mutation (14 available)
Tg(Alb-cre)21Mgn mutation (4 available)
phenotype observed in females
phenotype observed in males
N normal phenotype

Atherosclerosis lesion area is increased in Ldlrtm1Her/Ldlrtm1Her Nr1h3tm1.1Djm/Nr1h3tm1.1Djm Tg(Alb-cre)21Mgn/0 mice and TO901317 treatment reduces these lesions

homeostasis/metabolism
• by 4 weeks in mice fed a western diet
• mice fed a western diet and treated with T0901317 (an Lxr/Nr1h3 agonist) exhibit impaired reverse cholesterol transport compared with control mice
• by 4 weeks in mice fed a western diet
• in mice fed a western diet at the conclusion of the experiment
• mice fed a western diet and treated with T0901317 (an Lxr/Nr1h3 agonist) fail to exhibit a change in plasma lipid levels or an increase in fecal excretion of macrophage-derived sterols but an increase in hepatic sterols compared with control mice
• however, treatment with T0901317 reduces atherosclerosis as in controls

liver/biliary system
• in mice fed a western diet at the conclusion of the experiment

cardiovascular system
• mice fed a western diet exhibit increase in lesion size as detected by macrophage staining compared with control mice
• however, collagen staining or lesions is normal and treatment with T0901317 reduces atherosclerosis as in controls




Genotype
MGI:5607406
cn23
Allelic
Composition
Ldlrtm1Her/Ldlrtm1Her
Nr5a2tm2Sjns/Nr5a2tm2Sjns
Tg(Alb-cre)21Mgn/0
Genetic
Background
involves: 129/Sv * 129S7/SvEvBrd * C57BL/6 * C57BL/6J * DBA
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
Nr5a2tm2Sjns mutation (0 available); any Nr5a2 mutation (80 available)
Tg(Alb-cre)21Mgn mutation (4 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
N
• mice fed a high-cholesterol diet for 12 weeks exhibit a similar body and liver weight as single Ldlr homozygotes and do not develop increased atherosclerosis

digestive/alimentary system
• reverse cholesterol transport analysis indicates an increase in fecal sterol content compared to single Ldlr homozygote

homeostasis/metabolism
• reverse cholesterol transport analysis indicates an increase in fecal sterol content compared to single Ldlr homozygotes




Genotype
MGI:4367102
cx24
Allelic
Composition
Apobec1tm1Ddsn/Apobec1tm1Ddsn
Ldlrtm1Her/Ldlrtm1Her
Genetic
Background
B6.129-Apobec1tm1Ddsn Ldlrtm1Her
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Apobec1tm1Ddsn mutation (0 available); any Apobec1 mutation (12 available)
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
homeostasis/metabolism
• cholesterol is primarily in the LDL fraction as opposed to controls where it is predominantly in the HDL fraction
• shortened prothrombin times and activated thromboplastin times
• elevated thrombin and antithrombin levels

cardiovascular system
• plaque surface area increases from 24 to 72 weeks of age
• plaques composed of a fibrous cap over a foam cell core
• multilayered smooth muscle cell regions associated with collagen deposition at 24 weeks
• smooth muscle cell numbers diminished after 36 weeks
• collagen throughout the plaque core at 48 weeks




Genotype
MGI:4367109
cx25
Allelic
Composition
Apobec1tm1Ddsn/Apobec1tm1Ddsn
Fggtm1Fjc/Fggtm1Fjc
Ldlrtm1Her/Ldlrtm1Her
Genetic
Background
B6.129-Fggtm1Fjc Apobec1tm1Ddsn Ldlrtm1Her
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Apobec1tm1Ddsn mutation (0 available); any Apobec1 mutation (12 available)
Fggtm1Fjc mutation (0 available); any Fgg mutation (8 available)
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• display better survival to 3 weeks than do mice only deficient for Fgg

homeostasis/metabolism
• cholesterol is primarily in the LDL fraction as opposed to controls where it is predominantly in the HDL fraction
• thrombin and antithrombin levels very highly elevated

cardiovascular system
• plaque surface area increases beyond what is seen in double homozygotes lacking fibrinogen gamma chain deficiency
• fibrous cap thinner than is seen in double homozygotes lacking fibrinogen gamma chain deficiency
• collagen deposition in plaques develops more rapidly than in double homozygotes lacking fibrinogen gamma chain deficiency




Genotype
MGI:4946113
cx26
Allelic
Composition
Agtr1atm1Unc/Agtr1atm1Unc
Ldlrtm1Her/Ldlrtm1Her
Genetic
Background
B6.129-Ldlrtm1Her Agtr1atm1Unc
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Agtr1atm1Unc mutation (1 available); any Agtr1a mutation (15 available)
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• angiotensin II-treated mice fed a high-fat diet do not exhibit ascending aortic aneurysms unlike similarly treated Ldlrtm1Her homozygotes
• bone marrow transplant do not alter incidence of angiotensin II-induced ascending aortic aneurysms compared with similarly treated Ldlrtm1Her homozygotes




Genotype
MGI:3783838
cx27
Allelic
Composition
Apoa1tm1Unc/Apoa1tm1Unc
Ldlrtm1Her/Ldlrtm1Her
Genetic
Background
B6.129-Ldlrtm1Her Apoa1tm1Unc
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Apoa1tm1Unc mutation (2 available); any Apoa1 mutation (7 available)
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
homeostasis/metabolism
• when fed regular chow or a high fat diet for 16 weeks, serum triglyceride levels are increased compared to similarly treated Ldlrtm1Her homozygotes
• when fed a high fat diet, female mice exhibit a decrease in aortic cholesterol compared to similarly treated Ldlrtm1Her homozygotes
• however, aortic cholesterol levels in male mice fed a high fat diet are equivalent to in similarly treated Ldlrtm1Her homozygotes and female mice die before completion of the study
• when fed a high fat diet, mice exhibit a decrease in cholesterol content, specifically esterified cholesterol, in the adrenal gland compared to similarly treated Ldlrtm1Her homozygotes
• when mice are fed a high fat diet or regular chow, plasma cholesterol levels are decreased relative to similarly treated Ldlrtm1Her homozygotes
• unlike male Ldlrtm1Her homozygotes, plasma cholesterol levels fail to increased from weeks 8 to 16 on a high fat diet
• when fed a high fat diet, free cholesterol levels fail to increase as much as in similarly treated Ldlrtm1Her homozygotes
• unlike Ldlrtm1Her homozygotes, free cholesterol levels fail to increased from weeks 8 to 16 on a high fat diet
• when mice are fed a high fat diet or regular chow, esterified cholesterol levels are decreased relative to similarly treated Ldlrtm1Her homozygotes
• unlike male Ldlrtm1Her homozygotes, esterified cholesterol levels fail to increased from weeks 8 to 16 on a high fat diet
• when fed a high fat diet, mice exhibit a lesser increase in VLDL and LDL (3-fold) compared to Ldlrtm1Her homozygotes (6-fold)
• when fed regular chow or a high fat diet for 16 weeks, serum HDL levels are decreased compared to similarly treated Ldlrtm1Her homozygotes
• when fed a high fat diet, mice exhibit a lesser increase in VLDL and LDL (3-fold) compared to Ldlrtm1Her homozygotes (6-fold)
• when fed a high fat diet, mice exhibit a lesser increase in VLDL and LDL (3-fold) compared to Ldlrtm1Her homozygotes (6-fold)
• when fed a high fat diet, mice exhibit a reduced increase of 2.5-fold in liver cholesterol content compared to 11-fold in similarly treated Ldlrtm1Her homozygotes
• female mice develop skin lesions when fed a high fat diet
• total skin cholesterol content is increased 12- to 13-fold and 5.5-fold for free cholesterol compared to in Ldlrtm1Her homozygotes

liver/biliary system
• when fed a high fat diet, mice exhibit smaller diameter lipid droplets than in Ldlrtm1Her homozygotes
• when fed a high fat diet, mice exhibit an accumulation of inflammatory cells such as neutrophils and leukocytes around the hepatic vein unlike in Ldlrtm1Her homozygotes
• when fed a high fat diet, mice exhibit a reduced increase of 2.5-fold in liver cholesterol content compared to 11-fold in similarly treated Ldlrtm1Her homozygotes

endocrine/exocrine glands
• when fed a high fat diet, mice exhibit an decrease in cholesterol content, specifically esterified cholesterol, in the adrenal gland compared to in similarly treated Ldlrtm1Her homozygotes
• when fed a high fat diet, mice exhibit a severe depletion of cytoplasmic lipid droplets unlike in Ldlrtm1Her homozygotes

integument
• when fed a high fat diet, mice develop a thickened dermal layer with macrophage infiltrate and cholesterol deposits
• dermal thickening associated with a high fat diet is more severe in female mice than male mice
• when fed a high fat diet, female mice develop skin lesions beginning at 9 weeks with scratching and lesion areas that progress until animals cease eating and drinking
• skin lesions observed in mice fed a high fat diet are different than the non-fatal skin thickening observed in similarly treated Ldlrtm1Her homozygotes
• when fed a high fat diet, female mice exhibit severe ulcerations on thickened skin of the abdomen, neck and front limbs
• when fed a high fat diet, female mice develop pruritus (itching) and must be euthanized before the end of the 16 week study period




Genotype
MGI:3639678
cx28
Allelic
Composition
Gpr132tm1Witt/Gpr132tm1Witt
Ldlrtm1Her/Ldlrtm1Her
Genetic
Background
B6.129-Ldlrtm1Her Gpr132tm1Witt
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gpr132tm1Witt mutation (1 available); any Gpr132 mutation (8 available)
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• area in lesions occupied by macrophage is significantly increased
• reduced collagen content
• decreased apoptosis of macrophage in lesions

immune system
• decreased apoptosis of macrophage in atherosclerotic lesions

cellular
• decreased apoptosis of macrophage in atherosclerotic lesions

hematopoietic system
• decreased apoptosis of macrophage in atherosclerotic lesions




Genotype
MGI:4839086
cx29
Allelic
Composition
Ldlrtm1Her/Ldlrtm1Her
Tcra-Jtm1Tgi/Tcra-Jtm1Tgi
Genetic
Background
B6.129-Ldlrtm1Her Tcra-Jtm1Tgi
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
Tcra-Jtm1Tgi mutation (0 available); any Tcra-J mutation (0 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• lesions in the ascending aorta are reduced 20% in males and 28% in females relative to homozygous Ldlrtm1Her
• considerable reduction of lipid containing areas in the lesions
• less IFN-gamma in the lesions

immune system
• less IFN-gamma in atherosclerotic lesions




Genotype
MGI:4867042
cx30
Allelic
Composition
Ifngtm1Ts/Ifngtm1Ts
Ldlrtm1Her/Ldlrtm1Her
Genetic
Background
B6.129S7-Ldlrtm1Her Ifngtm1Ts
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ifngtm1Ts mutation (14 available); any Ifng mutation (27 available)
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• after 8 or 20 weeks on a cholesterol diet aortic lesion size is reduced compared to mice homozygous for Ldlrtm1Her alone

hematopoietic system
• increase in the proportion of T cells in the blood relative to controls homozygous for Ldlrtm1Her alone

immune system
• increase in the proportion of T cells in the blood relative to controls homozygous for Ldlrtm1Her alone




Genotype
MGI:4821420
cx31
Allelic
Composition
Cd1d1tm1Luc/Cd1d1tm1Luc
Ldlrtm1Her/Ldlrtm1Her
Genetic
Background
B6.129S-Cd1d1tm1Luc Ldlrtm1Her
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cd1d1tm1Luc mutation (2 available); any Cd1d1 mutation (11 available)
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• male mice after 4 weeks on the Western diet have lesions of small fatty streaks on the aorta were 40.4% smaller than Ldlrtm1Her
• Oil red O stained serial sections of 4-week-old mice fed a western diet, revealed had 31.7% less lipid staining than Ldlrtm1Her

homeostasis/metabolism
• slightly lower levels of very low density lipoprotein




Genotype
MGI:5008733
cx32
Allelic
Composition
Ctsstm1Hap/Ctsstm1Hap
Ldlrtm1Her/Ldlrtm1Her
Genetic
Background
B6.129S-Ctsstm1Hap Ldlrtm1Her
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ctsstm1Hap mutation (0 available); any Ctss mutation (14 available)
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• after 12 and 26 weeks on an atherogenic diet with decreased macrophage, leukocyte, and CD4+ T cells within lesions

immune system
• leukocytes exhibit impaired leukocyte transmigration compared with wild-type cells

homeostasis/metabolism
• when fed standard chow

muscle

cellular
• leukocytes exhibit impaired leukocyte transmigration compared with wild-type cells

hematopoietic system
• leukocytes exhibit impaired leukocyte transmigration compared with wild-type cells




Genotype
MGI:3721540
cx33
Allelic
Composition
Del(11Cxcl16-Zmynd15)1Ifc/Del(11Cxcl16-Zmynd15)1Ifc
Ldlrtm1Her/Ldlrtm1Her
Genetic
Background
B6.129S-Ldlrtm1Her Del(11Cxcl16-Zmynd15)1Ifc
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Del(11Cxcl16-Zmynd15)1Ifc mutation (0 available); any Del(11Cxcl16-Zmynd15)1Ifc mutation (0 available)
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• after 6 weeks, atherosclerosis lesions are 46% larger than in Ldlrtm1Her homozygotes
• after 10 weeks, atherosclerosis lesions are 27% and 56% (when assessed with serial sections of the aortic root stained with oil red O) larger than in Ldlrtm1Her homozygotes
• atherosclerotic lesions contain 80% more apoptotic cells than in Ldlrtm1Her homozygotes

cellular
• atherosclerotic lesions contain 80% more apoptotic cells than in Ldlrtm1Her homozygotes




Genotype
MGI:4367099
cx34
Allelic
Composition
Ldlrtm1Her/Ldlrtm1Her
Pfn1tm1Wit/Pfn1+
Genetic
Background
B6.129S-Ldlrtm1Her Pfn1tm1Wit
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
Pfn1tm1Wit mutation (0 available); any Pfn1 mutation (4 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• produced at a lower than expected ratio
• mice surviving to adulthood are healthy and normal regardless of diet fed

cardiovascular system
• lesions are reduced by 60% in males and 75% in females after 2 months on a high cholesterol diet as compared to Ldlr deficient mice
• less area in lesions is occupied by macrophage
• macrophage recruitment to lesion sites is reduced early in lesion formation

immune system
• macrophage recruitment to atherosclerotic lesion sites is reduced early in lesion formation
• ess area in lesions is occupied by macrophage

cellular
• macrophage recruitment to atherosclerotic lesion sites is reduced early in lesion formation
• ess area in lesions is occupied by macrophage

hematopoietic system
• macrophage recruitment to atherosclerotic lesion sites is reduced early in lesion formation
• ess area in lesions is occupied by macrophage




Genotype
MGI:3811068
cx35
Allelic
Composition
Ldlrtm1Her/Ldlrtm1Her
Serpine1tm1Mlg/Serpine1tm1Mlg
Genetic
Background
B6.129S-Serpine1tm1Mlg Ldlrtm1Her
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
Serpine1tm1Mlg mutation (4 available); any Serpine1 mutation (10 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• when fed a high fat Western diet for 6-30 weeks, mice develop atherosclerotic aortic lesions of the intimal and medial areas; incidence and severity are similar for Serpine1-deficient or transgenic Ldlr-deficient genotypes

homeostasis/metabolism
• mice develop severe cholesterolemia when receiving a high fat diet from 6 to 30 weeks (elevated levels are detected at 6, 15, and 30 weeks)




Genotype
MGI:4834596
cx36
Allelic
Composition
Ldlrtm1Her/Ldlrtm1Her
Vwftm1Wgr/Vwftm1Wgr
Genetic
Background
B6.129S-Vwftm1Wgr Ldlrtm1Her
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
Vwftm1Wgr mutation (1 available); any Vwf mutation (71 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• at 22 weeks, mice fed an atherogenic diet exhibit decreased atherosclerotic lesions size with fewer macrophages and reduced calcification compared with similarly treated Ldlrtm1Her homozygotes
• lesions in mice fed an atherogenic diet are uniformly distributed unlike in similarly treated Ldlrtm1Her homozygotes that exhibit formation hot spots
• however, mice exhibit normal atherosclerotic lesions at 37 weeks

immune system
• leukocyte rolling on a chow diet is decreased compared to in wild-type mice
• however, rolling on an atherogenic diet is normal

cellular
• leukocyte rolling on a chow diet is decreased compared to in wild-type mice
• however, rolling on an atherogenic diet is normal

hematopoietic system
• leukocyte rolling on a chow diet is decreased compared to in wild-type mice
• however, rolling on an atherogenic diet is normal




Genotype
MGI:5428446
cx37
Allelic
Composition
Ldlrtm1Her/Ldlrtm1Her
Tlr2tm1Kir/Tlr2tm1Kir
Genetic
Background
B6.129-Tlr2tm1Kir Ldlrtm1Her
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
Tlr2tm1Kir mutation (2 available); any Tlr2 mutation (20 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
homeostasis/metabolism
• reduced total plasma cholesterol on a high fat diet relative to Ldlrtm1Her/tm1Her controls

growth/size/body
• increased body weight on a high fat diet relative to Ldlrtm1Her/tm1Her controls (J:102502)

cardiovascular system
• aortic lesion area and aortic valve lesion volume are significantly reduced relative to Ldlrtm1Her/tm1Her controls (J:102502)
• less lipid accumulation in the lesser aortic curvature on a high fat diet than in Ldlrtm1Her/tm1Her controls (J:131783)

immune system
• leukocyte accumulation under the aortic endothelium is less than in Ldlrtm1Her/tm1Her controls
• significantly reduced response to LPS challenge

cellular
• leukocyte accumulation under the aortic endothelium is less than in Ldlrtm1Her/tm1Her controls

hematopoietic system
• leukocyte accumulation under the aortic endothelium is less than in Ldlrtm1Her/tm1Her controls




Genotype
MGI:3711207
cx38
Allelic
Composition
Ldlrtm1Her/Ldlrtm1Her
Mobq5CAST/Ei/Mobq5CAST/Ei
Genetic
Background
B6.CAST-Mobq5CAST/Ei
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
Mobq5CAST/Ei mutation (0 available); any Mobq5 mutation (0 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
homeostasis/metabolism
• decreased plasma cholesterol
• decreased unesterified cholesterol




Genotype
MGI:3711214
cx39
Allelic
Composition
Ldlrtm1Her/Ldlrtm1Her
Mobq6CAST/Ei/Mobq6CAST/Ei
Genetic
Background
B6.CAST-Mobq6CAST/Ei
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
Mobq6CAST/Ei mutation (0 available); any Mobq6 mutation (0 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
homeostasis/metabolism
• decreased obesity on a Western diet
• decreased unesterified cholesterol
• decreased plasma cholesterol
• improved glucose tolerance on a Western diet

cardiovascular system
• reduced aortic root lesion size (2-3 fold smaller)

behavior/neurological
• increased food efficiency

growth/size/body
• decreased obesity on a Western diet




Genotype
MGI:4947401
cx40
Allelic
Composition
Crptm1Hjf/Crptm1Hjf
Ldlrtm1Her/Ldlrtm1Her
Genetic
Background
B6.Cg-Crptm1Hjf Ldlrtm1Her
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Crptm1Hjf mutation (1 available); any Crp mutation (5 available)
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
homeostasis/metabolism
• in male mice at 20 weeks
• in male mice at 20 weeks
• in male mice at 20 weeks

cardiovascular system
• female mice exhibit an increase in macrophage content in lesions compared with Ldlrtm1Her homozygotes
• however, lesion size and susceptibility do not differ from Ldlrtm1Her homozygotes




Genotype
MGI:5286555
cx41
Allelic
Composition
Ins2Akita/Ins2Akita
Ldlrtm1Her/Ldlrtm1Her
Genetic
Background
B6.Cg-Ins2Akita Ldlrtm1Her
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ins2Akita mutation (13 available); any Ins2 mutation (23 available)
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• compared with Ldlrtm1Her homozygotes

homeostasis/metabolism
• severe compared with Ldlrtm1Her homozygotes
• in fasting mice compared with Ldlrtm1Her homozygotes
• in fasting mice compared with Ldlrtm1Her homozygotes
• in female mice compared with Ldlrtm1Her homozygotes
• 2-fold in male mice and 24% in female mice compared with Ldlrtm1Her homozygotes
• 7-fold in male mice and 1.8-fold in female mice compared with Ldlrtm1Her homozygotes
• in fasting mice compared with Ldlrtm1Her homozygotes
• compared with Ldlrtm1Her homozygotes

growth/size/body
• in male, but not female, mice at 20 weeks of age compared with Ldlrtm1Her homozygotes

cardiovascular system
• accelerated in mice fed a high-fat diet compared with similarly treated Ldlrtm1Her homozygotes




Genotype
MGI:3794980
cx42
Allelic
Composition
Ldlrtm1Her/Ldlrtm1Her
Scd1ab-2J/Scd1ab-2J
Genetic
Background
B6.Cg-Ldlrtm1Her Scd1ab-2J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
Scd1ab-2J mutation (2 available); any Scd1 mutation (13 available)
phenotype observed in females
phenotype observed in males
N normal phenotype

Adiposity in Ldlrtm1Her/Ldlrtm1Her and Scd1ab-2J/Scd1ab-2J Ldlrtm1Her/Ldlrtm1Her mice

adipose tissue
• when fed a western style diet for 12 weeks, male mice have smaller periepididymal fat pads than Ldlrtm1Her homozygotes
• when fed a western style diet for 12 weeks, female mice have smaller periuterine fat pads than Ldlrtm1Her homozygotes
• when fed a western style diet for 12 weeks, mice exhibit a 50% reduction in total fat mass compared to in Ldlrtm1Her homozygotes

homeostasis/metabolism
N
• when fed a western style diet for 12 weeks, mice are protected from impaired glucose tolerance, hyperglycemia, and increased plasma insulin levels observed in Ldlrtm1Her homozygotes
• when fed a western style diet for 12 weeks, mice exhibit a decrease in visceral and subcutaneous lipid compared to in Ldlrtm1Her homozygotes
• when fed a western style diet for 12 weeks, male mice exhibit a lower circulating cholesterol level than in Ldlrtm1Her homozygotes
• compared to in Ldlrtm1Her homozygotes when fed a western style diet for 12 weeks
• when fed a western style diet for 12 weeks, female mice exhibit a lower circulating triglyceride level than in Ldlrtm1Her homozygotes
• compared to in Ldlrtm1Her homozygotes when fed a western style diet for 12 weeks
• when fed a western style diet for 12 weeks, mice exhibit a 5-fold lower hepatic triglyceride level than in Ldlrtm1Her homozygotes

behavior/neurological
• when fed a western style diet for 12 weeks, mice consuming more food than Ldlrtm1Her homozygotes

growth/size/body
• when fed a western style diet for 12 weeks, mice gain less weight than Ldlrtm1Her homozygotes despite consuming more food

liver/biliary system
N
• when fed a western style diet for 12 weeks, mice are protected from the hepatic steatosis observed in Ldlrtm1Her homozygotes
• when fed a western style diet for 12 weeks, mice exhibit a 5-fold lower hepatic triglyceride level than in Ldlrtm1Her homozygotes




Genotype
MGI:3794981
cx43
Allelic
Composition
Ldlrtm1Her/Ldlrtm1Her
Scd1ab-J/Scd1ab-J
Genetic
Background
B6.Cg-Ldlrtm1Her Scd1ab-J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
Scd1ab-J mutation (0 available); any Scd1 mutation (13 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
adipose tissue
• when fed a western style diet for 12 weeks, male mice have smaller periepididymal fat pads than Ldlrtm1Her homozygotes
• when fed a western style diet for 12 weeks, female mice have smaller periuterine fat pads than Ldlrtm1Her homozygotes
• when fed a western style diet for 12 weeks, mice exhibit a 50% reduction in total fat mass compared to in Ldlrtm1Her homozygotes

homeostasis/metabolism
N
• when fed a western style diet for 12 weeks, mice are protected from impaired glucose tolerance, hyperglycemia and increased plasma insulin levels observed in Ldlrtm1Her homozygotes
• when fed a western style diet for 12 weeks, mice exhibit a decrease in visceral and subcutaneous lipid compared to in Ldlrtm1Her homozygotes
• when fed a western style diet for 12 weeks, male mice exhibit a lower circulating cholesterol level than in Ldlrtm1Her homozygotes
• compared to in Ldlrtm1Her homozygotes when fed a western style diet for 12 weeks
• when fed a western style diet for 12 weeks, mice exhibit a 5-fold lower hepatic triglyceride level than in Ldlrtm1Her homozygotes
• when fed a western style diet for 12 weeks, female mice exhibit a lower circulating triglyceride level than in Ldlrtm1Her homozygotes
• when fed a western style diet for 12 weeks, female mice exhibit a lower circulating triglyceride level than in Ldlrtm1Her homozygotes
• compared to in Ldlrtm1Her homozygotes when fed a western style diet for 12 weeks
• when fed a western style diet for 12 weeks, mice exhibit a 5-fold lower hepatic triglyceride level than in Ldlrtm1Her homozygotes

behavior/neurological
• when fed a western style diet for 12 weeks, mice consuming more food than Ldlrtm1Her homozygotes

growth/size/body
• when fed a western style diet for 12 weeks, mice gain less weight than Ldlrtm1Her homozygotes despite consuming more food

liver/biliary system
N
• when fed a western style diet for 12 weeks, mice are protected from the hepatic steatosis observed in Ldlrtm1Her homozygotes
• when fed a western style diet for 12 weeks, mice exhibit a 5-fold lower hepatic triglyceride level than in Ldlrtm1Her homozygotes




Genotype
MGI:3810981
cx44
Allelic
Composition
Ldlrtm1Her/Ldlrtm1Her
Tg(CMV-Serpine1)1Dgi/0
Genetic
Background
B6.Cg-Ldlrtm1Her Tg(CMV-Serpine1)1Dgi
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
Tg(CMV-Serpine1)1Dgi mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• when fed a high fat Western diet for 6-30 weeks, mice develop atherosclerotic aortic lesions of the intimal and medial areas; incidence and severity are similar for Serpine1-deficient or transgenic Ldlr-deficient genotypes

homeostasis/metabolism
• mice develop severe cholesterolemia receiving a high fat diet from 6 to 30 weeks (elevated levels are detected at 6, 15, and 30 weeks)




Genotype
MGI:3622658
cx45
Allelic
Composition
Ldlrtm1Her/Ldlr+
Lepob/Lepob
Genetic
Background
B6.Cg-Lepob Ldlrtm1Her
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
Lepob mutation (6 available); any Lep mutation (14 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
homeostasis/metabolism
• age dependent increase in cholesterol levels between 1 and 4 months of age, with maximal values at 3-4 months of age (282 mg/dl vs 81 md/dl in wild-type) that are maintained thereafter
• slightly elevated at 3 and 8 months of age
• livers exhibit slightly, but significantly, higher levels of cholesterol
• livers exhibit about 10x higher levels of triglyceride, however no increase in plasma triglyceride levels

liver/biliary system
• livers exhibit slightly, but significantly, higher levels of cholesterol
• livers exhibit about 10x higher levels of triglyceride, however no increase in plasma triglyceride levels




Genotype
MGI:3622656
cx46
Allelic
Composition
Ldlrtm1Her/Ldlrtm1Her
Lepob/Lepob
Genetic
Background
B6.Cg-Lepob Ldlrtm1Her
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
Lepob mutation (6 available); any Lep mutation (14 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• extensive atherosclerotic lesions throughout the aorta by 6 months of age

homeostasis/metabolism
• plasma contains severely elevated and broadened lipoprotein peak, ranging from VLDL/LDL-sized particles to LDL-sized particles
• age dependent increase in cholesterol levels between 1 and 4 months of age, with maximal values at 3-4 months of age (1715 mg/dl vs. 81 mg/dl in wild-type), followed by a gradual decrease by 8 months
• fasting, diet restriction, and low-level leptin treatment only slightly lowers plasma cholesterol levels
• elevated at 3 and 8 months of age
• age dependent increase in triglyceride levels between 1 and 4 months of age, with maximal values at 3-4 months of age, followed by a gradual decrease by 8 months
• fasting, diet restriction, and low-level leptin treatment significantly lowers plasma triglyceride levels
• livers exhibit slightly, but significantly, higher levels of cholesterol
• livers exhibit about 10x higher levels of triglyceride

liver/biliary system
• livers exhibit slightly, but significantly, higher levels of cholesterol
• livers exhibit about 10x higher levels of triglyceride

Mouse Models of Human Disease
OMIM ID Ref(s)
Hypercholesterolemia, Familial 143890 J:72027




Genotype
MGI:4367224
cx47
Allelic
Composition
Apoetm1Unc/Apoetm1Unc
Ldlrtm1Her/Ldlrtm1Her
Genetic
Background
involves: 129P2/OlaHsd * 129S7/SvEvBrd
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Apoetm1Unc mutation (22 available); any Apoe mutation (68 available)
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
homeostasis/metabolism
• plasma cholesterol on a high fat diet reaches 4120 mg/dl
• triglyceride levels are unaffected by diet




Genotype
MGI:4367222
cx48
Allelic
Composition
Ldlrtm1Her/Ldlrtm1Her
Lipctm1Unc/Lipctm1Unc
Genetic
Background
involves: 129P2/OlaHsd * 129S7/SvEvBrd * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
Lipctm1Unc mutation (4 available); any Lipc mutation (16 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
homeostasis/metabolism
• levels in response to ACTH are significantly reduced
• levels markedly increased
• primarily in HDL and LDL fractions




Genotype
MGI:4367112
cx49
Allelic
Composition
Ldlrtm1Her/Ldlrtm1Her
Mapkapk2tm1Mgl/Mapkapk2tm1Mgl
Genetic
Background
involves: 129P2/OlaHsd * 129S7/SvEvBrd * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
Mapkapk2tm1Mgl mutation (0 available); any Mapkapk2 mutation (20 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• reduced development of atherosclerosis after 8-16 weeks on an atherogenic diet

hematopoietic system
• reduced in number among peritoneal macrophage in mice fed an atherogenic diet for 10 weeks
• decreased macrophage content in longitudinal sections of the aortic arch

homeostasis/metabolism
• increased plasma cholesterol relative to mice deficient only in Ldlr
• increased non-HDL cholesterol relative to mice deficient only in Ldlr

immune system
• reduced in number among peritoneal macrophage in mice fed an atherogenic diet for 10 weeks
• decreased macrophage content in longitudinal sections of the aortic arch

cellular
• reduced in number among peritoneal macrophage in mice fed an atherogenic diet for 10 weeks




Genotype
MGI:4358708
cx50
Allelic
Composition
Lcattm1Hgc/Lcattm1Hgc
Ldlrtm1Her/Ldlrtm1Her
Genetic
Background
involves: 129P2/OlaHsd * 129S7/SvEvBrd * C57BL/6 * DBA
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Lcattm1Hgc mutation (0 available); any Lcat mutation (8 available)
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
homeostasis/metabolism
N
• unlike Lcat single mutants, the free cholesterol/esterfied cholesterol ratio is not significantly different from controls
• fasting glucose levels are 31% lower than the single mutant controls
• fasting insulin levels are 42% lower than the single mutant controls
• increase in APOB-100
• about a 2 fold decrease in all plasma lipid constituents compared to mice homozygous null for Lcat and Apoe (J:75567)
• unlike in Ldlr single mutants, the long chain PUFA, 20:4, 20:5 n-3 and 22:6 n-3 esters are absent (J:75567)
• 8 fold increase in triglyceride production rate compared to single mutant controls (J:89015)
• increase in plasma triglyceride levels compared to Ldlr single mutants (J:75567)
• 1.7 fold increase in fasting triglyceride levels compared to single mutant littermate controls (J:89015)
• most of the excess triglycerides are concentrated in the VLDL fractions; however, both the LDL and IDL fractions are enriched for triglycerides (J:89015)
• compared to Ldlr single mutants the APOB lipoprotein cholesterol ester composition has increases in the 16:0, 18:0, and 18:1 esters and decreases in the 18:2 esters (J:75567)
• there is a 7 fold increase in the ratio of saturated + monounsaturated/polyunsaturated cholesterol ester fatty acid species compared to controls (J:75567)
• total plasma free cholesterol : cholesterol ester ratio is significantly increased compared to controls (J:89015)
• no detectable HDL (J:75567)
• severely reduced (J:89015)
• decrease in the LDL/IDL fractions compared to controls
• increase in free cholesterol level compared to Ldlr single mutants
• increase in the cholesterol level in the VLDL fraction (J:89015)
• decrease in post heparin lipase activity




Genotype
MGI:3789482
cx51
Allelic
Composition
Apoetm1Unc/Apoetm1Unc
Ldlrtm1Her/Ldlrtm1Her
Genetic
Background
involves: 129P2/OlaHsd * 129S7/SvEvBrd * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Apoetm1Unc mutation (22 available); any Apoe mutation (68 available)
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• two out of 8 hypoxic hearts show thrombosis as well as atherosclerotic plaques
• elevated STU segment as a result of induced hypoxia
• noticeable at 16% oxygen concentration
• ECG is corrected by 10 minutes after restoration of normal oxygen
• hypoxia response unaffected by treatment with a thrombin inhibitor
• mice treated with a thrombin inhibitor display lower Troponin-T levels and fewer fibrinogen + cells than untreated mice 48 hours after hypoxia
• in mice treated with a thrombin inhibitor 48 hours after hypoxia
• infarctions are irregular in shape and subendocardial or intramural

homeostasis/metabolism
• mice treated with a thrombin inhibitor display lower Troponin-T levels and fewer fibrinogen + cells than untreated mice 48 hours after hypoxia
• in mice treated with a thrombin inhibitor 48 hours after hypoxia
• infarctions are irregular in shape and subendocardial or intramural

nervous system
• elevated cholesterol in the exofacial leaflet of the synaptic plasma membrane but not so high as for Ldlr deficient mice with normal Apoe alleles
• cholesterol levels in the cytofacial leaflet are reduced
• cholesterol/phospholipid ratio in the synaptic plasma membrane is elevated but not so much as for Ldlr deficient mice with normal Apoe alleles




Genotype
MGI:4367117
cx52
Allelic
Composition
Ddr1tm1Wfv/Ddr1tm1Wfv
Ldlrtm1Her/Ldlrtm1Her
Genetic
Background
involves: 129S1/Sv * 129S7/SvEvBrd * 129X1/SvJ
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ddr1tm1Wfv mutation (0 available); any Ddr1 mutation (7 available)
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• pronounced reduction in atherosclerotic plaques after 12 and 24 weeks on a high fat diet
• 50% reduction in plaque area in the descending aorta at 12 weeks
• 60% reduction in plaque area in the descending aorta at 24 weeks
• total collagen and elastin in plaques is elevated at 12 weeks
• total collagen and elastin in plaques is at control levels at 24 weeks
• increased synthesis and decreased degradation of collagen and elastin at 12 weeks

hematopoietic system
• few macrophage in plaques at 12 weeks
• macrophage numbers in plaques similar to controls at 24 weeks

immune system
• few macrophage in plaques at 12 weeks
• macrophage numbers in plaques similar to controls at 24 weeks




Genotype
MGI:4943544
cx53
Allelic
Composition
Ldlrtm1Her/Ldlrtm1Her
Pcsk9tm1.2Prat/Pcsk9tm1.2Prat
Genetic
Background
involves: 129S1/Sv * 129S7/SvEvBrd * 129X1/SvJ
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
Pcsk9tm1.2Prat mutation (0 available); any Pcsk9 mutation (10 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
homeostasis/metabolism
• plasma levels are increased 14.8 fold relative to controls
• plasma HDL levels are unaffected




Genotype
MGI:3629493
cx54
Allelic
Composition
Cxcl1tm1Wabo/Cxcl1tm1Wabo
Ldlrtm1Her/Ldlrtm1Her
Genetic
Background
involves: 129S1/Sv * 129S7/SvEvBrd * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cxcl1tm1Wabo mutation (0 available); any Cxcl1 mutation (4 available)
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• surface lesions in aortas are significantly smaller than those in Ldlr homozygous, Cxcl wild-type littermates (14.2% vs 17.1%)
• lesion area in the aoritic valve is smaller than in littermates (186,423 micron2 vs 238636 micron2
• there is less macrophage accumualation in lesions of double knockouts than in control single mutants
• double knockouts are less susceptible than Ldlr single knockouts




Genotype
MGI:3833895
cx55
Allelic
Composition
Esr1tm1.1Mma/Esr1tm1.1Mma
Ldlrtm1Her/Ldlrtm1Her
Genetic
Background
involves: 129S2/SvPas * 129S7/SvEvBrd
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Esr1tm1.1Mma mutation (0 available); any Esr1 mutation (22 available)
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
homeostasis/metabolism
• estrogen fails to prevent fatty streak deposits in the aorta of these mice as it does in Ldlr null homozygote controls

cardiovascular system
• lacking functional Ldlr receptors makes this mice prone to developing atherosclerosis




Genotype
MGI:4439281
cx56
Allelic
Composition
Itgb3tm1Hyn/Itgb3tm1Hyn
Ldlrtm1Her/Ldlrtm1Her
Genetic
Background
involves: 129S2/SvPas * 129S7/SvEvBrd * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Itgb3tm1Hyn mutation (5 available); any Itgb3 mutation (23 available)
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• at 16 weeks of age after 10 weeks on a western style diet only 52% of mice survive compared to 100% survival for diet matched Ldlr single mutants
• no increase in mortality is seen in mice kept on a standard chow diet
• at 16 weeks of age after 10 weeks on a western style diet only 52% of mice survive compared to 100% survival for diet matched Ldlr single mutants

homeostasis/metabolism
• the level after 10 weeks on a western diet is higher than in diet matched Ldlr single mutants
• cholesterol levels after 6 and 10 weeks on a western diet are lower than in diet matched Ldlr single mutants

cardiovascular system
• on a western style diet, lesions are 2.0 fold greater at the arch and 4.1 fold greater at the thoracic aorta compared to diet matched Ldlr single mutants




Genotype
MGI:4367211
cx57
Allelic
Composition
Alox15tm1Fun/Alox15tm1Fun
Ldlrtm1Her/Ldlrtm1Her
Genetic
Background
involves: 129S2/SvPas * 129S7/SvEvBrd * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Alox15tm1Fun mutation (3 available); any Alox15 mutation (17 available)
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• reduced atherosclerotic lesions when fed a high fat diet for 3 or 9 weeks




Genotype
MGI:3850551
cx58
Allelic
Composition
Apobec1tm1Chan/Apobec1tm1Chan
Ldlrtm1Her/Ldlrtm1Her
Genetic
Background
involves: 129S4/SvJae * 129S7/SvEvBrd
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Apobec1tm1Chan mutation (0 available); any Apobec1 mutation (12 available)
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
homeostasis/metabolism
• circulating VLDL/LDL cholesterol levels are increased compared to in Ldlrtm1Her homozygotes, Apobec1tm1Chan homozygotes, and wild-type mice
• when fed a chow diet or Western-type diet for 2 and 4 weeks, mice exhibit increased serum cholesterol levels compared with Ldlrtm1Her homozygotes, Apobec1tm1Chan homozygotes, and wild-type mice
• when fed a Western-type diet for 14 weeks, mice exhibit higher serum cholesterol levels compared with Ldlrtm1Her homozygotes
• when fed a Western-type diet for 2 weeks, serum triglyceride levels are less than in Ldlrtm1Her homozygotes
• when fed a chow diet or a high fat diet for 2 and 4 weeks, mice exhibit a decrease in serum triglyceride levels compared with Apobec1tm1Chan homozygotes and wild-type mice




Genotype
MGI:3620575
cx59
Allelic
Composition
Icam1tm1Jcgr/Icam1tm1Jcgr
Ldlrtm1Her/Ldlrtm1Her
Genetic
Background
involves: 129S4/SvJae * 129S7/SvEvBrd
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Icam1tm1Jcgr mutation (3 available); any Icam1 mutation (15 available)
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
hematopoietic system
• neutrophil counts are increased when mice have been on an 8-week cholesterol-enriched diet compared to homozygous Ldlr mice, however develop aortic lesions to the same extent as homozygous Ldlr mice

immune system
• neutrophil counts are increased when mice have been on an 8-week cholesterol-enriched diet compared to homozygous Ldlr mice, however develop aortic lesions to the same extent as homozygous Ldlr mice




Genotype
MGI:4418562
cx60
Allelic
Composition
Ccl2tm1Rol/Ccl2tm1Rol
Ldlrtm1Her/Ldlrtm1Her
Genetic
Background
involves: 129S4/SvJae * 129S7/SvEvBrd * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ccl2tm1Rol mutation (1 available); any Ccl2 mutation (12 available)
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• when fed a high fat diet, mice exhibit decreased incidence of atherosclerosis, lipid deposition, and macrophage infiltration of aortic arches compared with similarly treated Ldlrtm1Her homozygotes

homeostasis/metabolism
• compared to in Ldlrtm1Her homozygotes
• compared to in Ldlrtm1Her homozygotes




Genotype
MGI:3620574
cx61
Allelic
Composition
Icam1tm1Jcgr/Icam1tm1Jcgr
Ldlrtm1Her/Ldlrtm1Her
Vcam1tm1Dmil/Vcam1tm1Dmil
Genetic
Background
involves: 129S4/SvJae * 129S7/SvEvBrd * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Icam1tm1Jcgr mutation (3 available); any Icam1 mutation (15 available)
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
Vcam1tm1Dmil mutation (0 available); any Vcam1 mutation (16 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• after an 8-week cholesterol-enriched diet, whole aorta and arch lesion area is reduced by 31% and 45%, respectively, compared to double homozygous Icam1 and Ldlr mice, and by 48% and 38%, respectively, compared to single homozygous Ldlr mutant mice

homeostasis/metabolism
• after an 8-week cholesterol-enriched diet, develop a less severe hypercholesterolemia than single homozygous Ldlr mutant mice

immune system
• total leukocyte counts are increased when mice have been on an 8-week cholesterol-enriched diet compared to homozygous Ldlr mutant mice
• neutrophil counts are increased when mice have been on an 8-week cholesterol-enriched diet compared to homozygous Ldlr mutant mice
• lymphocyte counts are increased when mice have been on an 8-week cholesterol-enriched diet compared to homozygous Ldlr mutant mice
• monocyte counts are increased when mice have been on an 8-week cholesterol-enriched diet compared to homozygous Ldlr mutant mice

hematopoietic system
• total leukocyte counts are increased when mice have been on an 8-week cholesterol-enriched diet compared to homozygous Ldlr mutant mice
• neutrophil counts are increased when mice have been on an 8-week cholesterol-enriched diet compared to homozygous Ldlr mutant mice
• lymphocyte counts are increased when mice have been on an 8-week cholesterol-enriched diet compared to homozygous Ldlr mutant mice
• monocyte counts are increased when mice have been on an 8-week cholesterol-enriched diet compared to homozygous Ldlr mutant mice




Genotype
MGI:3620572
cx62
Allelic
Composition
Ldlrtm1Her/Ldlrtm1Her
Vcam1tm1Dmil/Vcam1tm1Dmil
Genetic
Background
involves: 129S4/SvJae * 129S7/SvEvBrd * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
Vcam1tm1Dmil mutation (0 available); any Vcam1 mutation (16 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• develop fewer early foam cell lesions in the aorta than homozygous Ldlr mice after an 8 week cholesterol-enriched diet, however cholesterol levels, lipoprotein profiles and leukocyte numbers are similar to wild-type




Genotype
MGI:4367223
cx63
Allelic
Composition
Ldlrtm1Her/Ldlrtm1Her
Pparatm1Gonz/Pparatm1Gonz
Genetic
Background
involves: 129S4/SvJae * 129S7/SvEvBrd * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
Pparatm1Gonz mutation (3 available); any Ppara mutation (16 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
homeostasis/metabolism
N
• fasting glucose levels and insulin levels are not increased after dexamethasone treatment

growth/size/body

adipose tissue
• after dexamethasone treatment




Genotype
MGI:4367101
cx64
Allelic
Composition
Apobec1tm1Ddsn/Apobec1tm1Ddsn
Ldlrtm1Her/Ldlrtm1Her
Genetic
Background
involves: 129S4/SvJae * 129S7/SvEvBrd * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Apobec1tm1Ddsn mutation (0 available); any Apobec1 mutation (12 available)
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
homeostasis/metabolism
• total cholesterol levels are higher in males than females
• on a high fat diet total cholesterol reaches 1600 mg/dl by 8 weeks
• elevated in both sexes but higher in males than females

cardiovascular system
• extensive lesions along the length of the aorta at 8 months
• lesions are somewhat more extensive in males but otherwise the sexes are similar
• on a high fat diet lesions cover 9% of the aorta surface by 8 weeks

Mouse Models of Human Disease
OMIM ID Ref(s)
Hypercholesterolemia, Familial 143890 J:49125




Genotype
MGI:3761833
cx65
Allelic
Composition
Ldlrtm1Her/Ldlrtm1Her
Soat1tm1Far/Soat1tm1Far
Genetic
Background
involves: 129S4/SvJae * 129S7/SvEvBrd * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
Soat1tm1Far mutation (3 available); any Soat1 mutation (24 available)
phenotype observed in females
phenotype observed in males
N normal phenotype

Soat1tm1Far/Soat1tm1Far Ldlrtm1Her/Ldlrtm1Her mice fed an atherogenic diet for 2 months display severe xanthomatosis

homeostasis/metabolism
• serum total cholesterol levels in mutants fed the atherogenic diet for 2 months are lower than in single Ldlr homozygotes
• skin of mutants fed an atherogenic diet shows edematous lesions
• double mutants fed an atherogenic diet for 2 months develop severe dermal xanthomatosis; skin shows diffuse deposition of cholesterol crystals in both the reticular and papillary dermis

cardiovascular system
• mutants fed a Western diet develop atherosclerotic lesions
• aortic lesion composition differs from that in Apoe homozygotes, with lower neutral lipid content and fewer cholesterol crystals

immune system
• severe, pleomorphic inflammatory reaction accompanies the cholesterol deposition in atherogenic diet fed mutants, however the features are different from those in double Soat1 and Apoe mutants
• inflammatory cells are predominantly macrophages, lymphocytes, and plasma cells, with fewer neutrophils than in double Soat1 and Apoe mutants

nervous system
• mutants fed an atherogenic diet exhibit crystalline cholesterol deposits in the brains, particularly near the choroid plexus and in the cerebellum
• mutants fed an atherogenic diet exhibit crystalline cholesterol deposits in the cerebellum

integument
• skin of mutants fed an atherogenic diet shows edematous lesions
• severe, pleomorphic inflammatory reaction accompanies the cholesterol deposition in atherogenic diet fed mutants, however the features are different from those in double Soat1 and Apoe mutants
• inflammatory cells are predominantly macrophages, lymphocytes, and plasma cells, with fewer neutrophils than in double Soat1 and Apoe mutants
• diffuse deposition of cholesterol crystals in the reticular and papillary dermis of mutants fed an atherogenic diet
• skin lesions are seen only when mutants are fed a Western diet and not when fed a chow diet
• mutants fed an atherogenic diet, but not a chow diet, develop diffuse, massive thickening of the skin compared to single Ldlr mutants
• however, do not develop skin excoriations, hair loss or pruritic skin




Genotype
MGI:3623224
cx66
Allelic
Composition
Ldlrtm1Her/Ldlrtm1Her
Scarb1tm1Dhu/Scarb1tm1Dhu
Genetic
Background
involves: 129S4/SvJae * BALB/c * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
Scarb1tm1Dhu mutation (0 available); any Scarb1 mutation (21 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• lesion size is significantly increased in males compared to LDL-receptor controls




Genotype
MGI:3849585
cx67
Allelic
Composition
Angptl3tm1Lex/Angptl3tm1Lex
Ldlrtm1Her/Ldlrtm1Her
Genetic
Background
involves: 129S5/SvEvBrd * 129S7/SvEvBrd * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Angptl3tm1Lex mutation (2 available); any Angptl3 mutation (15 available)
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
homeostasis/metabolism
• serum cholesterol levels are 60% lower than in Ldlr null homozygote controls
• serum triglyceride levels are 86% lower than in Ldlr null homozygote controls




Genotype
MGI:3719026
cx68
Allelic
Composition
Ldlrtm1Her/Ldlrtm1Her
Tbx21tm1Glm/Tbx21tm1Glm
Genetic
Background
involves: 129S6/SvEvTac * 129S7/SvEvBrd * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
Tbx21tm1Glm mutation (4 available); any Tbx21 mutation (17 available)
phenotype observed in females
phenotype observed in males
N normal phenotype

Phenotype of aortic arch atherosclerotic lesions in Tbx21tm1Glm/Tbx21tm1Glm Ldlrtm1Her/Ldlrtm1Her and Ldlrtm1Her/Ldlrtm1Her mice

cardiovascular system
• atherosclerosis in both aortic arch and descending aorta is significantly reduced compared with Ldlrtm1Her homozygotes and lesions have less smooth muscle content
• males have an 81% reduction in the aortic arch intimal area of lesions and 49% reduction in descending aorta compared with control males, however no differences seen in females
• after 8 weeks of proatherogenic diet, intimal smooth muscle cell content is lower in aortic arch than in controls, indicating a delay in evolution of lesions

homeostasis/metabolism
• hypercholesterolemic; cholesterol levels are no different from those in Ldlrtm1Her homozygotes

immune system
• significant reduction of total IgG2a compared to Ldlrtm1Her homozygotes; IgG2a titers to both MDA-LDL and CuOx-LDL are reduced or absent
• IgG1 titers to both antigens (MDA-LDL and CuOx-LDL) are reduced despite similar total levels of IgG1
• exhibit a more than 2.5-fold increase in the titer of atheroprotective E06 natural IgM antibodies compared to Ldlrtm1Her homozygotes
• unlike CD4+ T cells in Ldlrtm1Her homozygotes on a high cholesterol diet, T cells do not produce significant amounts of IFN-gamma upon polyclonal stimulation with anti-CD3 and upon stimulation with plaque antigen hsp60
• on a high cholesterol diet, higher levels of IL-4, IL-5, and IL-10 are produced upon anti-CD3 stimulation and higher levels of IL-5 and IL-10 upon hsp60 stimulation of T cells compared to T cells from Ldlrtm1Her homozygotes

hematopoietic system
• significant reduction of total IgG2a compared to Ldlrtm1Her homozygotes; IgG2a titers to both MDA-LDL and CuOx-LDL are reduced or absent
• IgG1 titers to both antigens (MDA-LDL and CuOx-LDL) are reduced despite similar total levels of IgG1
• exhibit a more than 2.5-fold increase in the titer of atheroprotective E06 natural IgM antibodies compared to Ldlrtm1Her homozygotes




Genotype
MGI:3833896
cx69
Allelic
Composition
Esr1tm1Arnal/Esr1tm1Arnal
Ldlrtm1Her/Ldlrtm1Her
Genetic
Background
involves: 129S7/SvEvBrd
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Esr1tm1Arnal mutation (0 available); any Esr1 mutation (22 available)
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
homeostasis/metabolism
• estrogen fails to prevent fatty streak deposits in the aorta of these mice as it does in Ldlr null homozygote controls

cardiovascular system
• lacking functional Ldlr receptors makes these mice prone to developing atherosclerosis




Genotype
MGI:3772340
cx70
Allelic
Composition
Ldlrtm1Her/Ldlrtm1Her
Olr1tm1Meht/Olr1tm1Meht
Genetic
Background
involves: 129S7/SvEvBrd * 129X1/SvJ * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
Olr1tm1Meht mutation (0 available); any Olr1 mutation (13 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
homeostasis/metabolism
• when fed a high-cholesterol diet, NMR proton spectra of lipids indicates a level of photon intensity for HDL of 0.77 compared to 0.83 in wild-type mice
• when fed a high cholesterol diet, NMR proton spectra of lipids indicates a level of photon intensity for LDL of 0.88 compared to 0.84 in wild-type mice
• when fed a high cholesterol diet, NMR proton spectra of lipids indicates a level of photon intensity for VLDL of 0.92 compared to 0.88 in wild-type mice
• IL-10 levels are increased compared to in Ldlrtm1Her homozygotes

cardiovascular system
• mice exhibit less intimal thickening and sudanophilic areas than in Ldlrtm1Her homozygotes
• mice do not exhibit endothelial disruption as in Ldlrtm1Her homozygotes

immune system
• IL-10 levels are increased compared to in Ldlrtm1Her homozygotes




Genotype
MGI:3785901
cx71
Allelic
Composition
Ldlrtm1Her/Ldlrtm1Her
Npc1m1N/Npc1m1N
Genetic
Background
involves: 129S7/SvEvBrd * BALB/c
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ldlrtm1Her mutation (19 available); any Ldlr mutation (48 available)
Npc1m1N mutation (2 available); any Npc1 mutation (37 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• mice die between 80 and 100 days of age from neurodegenerative disease

nervous system
• mice have decreased levels of total cholesterol in the brain
• reduced in size by 50% at 11 weeks of age
• glial cells reduced by 44%
• axonal swelling by 11 weeks of age
• small reduction in Purkinje cell numbers in the cerebellar hemispheres at 3 weeks of age
• reduction in numbers increases with age
• glial cells in the corpus callosum reduced by 44%

homeostasis/metabolism
• mice have decreased levels of total cholesterol in the brain
• mice have increased cholesterol levels in the liver, spleen, intestine and lung to a greater extant than that observed in Npc1 mutants on a wild-type background
• plasma LDL levels are increased 15-fold in these mice