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All mouse models of cerebral degeneration with phenotypic similarity to the human disease
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| Disease Term | Allelic Composition | Genetic Background | Reference | Phenotypes | ||||
  | adrenoleukodystrophy | Abcd1tm1Kan/Abcd1tm1Kan | involves: 129S1/Sv * 129X1/SvJ * C57BL/6J | J:44812 | View | |||
| adrenoleukodystrophy | Abcd1tm1Kan/Y | involves: 129S1/Sv * 129X1/SvJ * C57BL/6J | J:44812 | View | ||||
| adrenoleukodystrophy |
Abcd1tm1Kan/Y Gt(ROSA)26Sortm2.1(CAG-ELOVL1)Geno/Gt(ROSA)26Sor+ Cnptm1(cre)Kan/Cnp+ |
involves: 129 * 129S1/Sv * 129X1/SvJ * C57BL/6J | J:257393 | View | ||||
| adrenoleukodystrophy | Abcd1tm1Kds/Abcd1tm1Kds | either: (involves: 129S4/SvJae * C57BL/6) or (involves: 129/Sv * 129S4/SvJae * C57BL/6) | J:42479 | View | ||||
| adrenoleukodystrophy | Abcd1tm1Kds/Abcd1tm1Kds | involves: 129/Sv * 129S4/SvJae * C57BL/6J | J:75388 | View | ||||
| adrenoleukodystrophy | Abcd1tm1Kds/Y | either: (involves: 129S4/SvJae * C57BL/6) or (involves: 129/Sv * 129S4/SvJae * C57BL/6) | J:42479 | View | ||||
| adrenoleukodystrophy | Abcd1tm1Kds/Y | involves: 129S4/SvJae * C57BL/6J | J:94583 | View | ||||
| adrenoleukodystrophy | Abcd1tm1Ymd/Abcd1tm1Ymd | involves: 129S/SvEv * C57BL/6J * DBA/2J | J:40230 | View | ||||
| adrenoleukodystrophy | Abcd1tm1Ymd/Y | involves: 129S/SvEv * C57BL/6J * DBA/2J | J:40230 | View | ||||
| adult-onset autosomal dominant demyelinating leukodystrophy | Tg(Lmnb1)1Yfu/0 | involves: C57BL/6J | J:197168 | View | ||||
| Alexander disease | Gfaptm2Mes/Gfap+ | involves: 129S6/SvEvTac * 129S7/SvEvBrd * FVB/N | J:114689 | View | ||||
| Alexander disease |
Gfaptm2Mes/Gfap+ Tg(GFAP)10Mes/0 |
involves: 129S6/SvEvTac * 129S7/SvEvBrd * FVB/N | J:114689 | View | ||||
| Alexander disease |
Gfaptm2Mes/Gfap+ Tg(GFAP)10Mes/0 |
involves: 129S7/SvEvBrd * FVB/N | J:146191 | View | ||||
| Alexander disease | Gfaptm3Mes/Gfap+ | involves: 129S6/SvEvTac * 129S7/SvEvBrd * FVB/N | J:114689 | View | ||||
| CADASIL 1 |
Gt(ROSA)26Sortm2(NOTCH3*C455R)Sat/Gt(ROSA)26Sor+ Notch3Gt(PST033)Byg/Notch3Gt(PST033)Byg Tg(Tagln-cre)1Her/0 |
involves: 129 * C57BL/6 * SJL | J:171887 | View | ||||
| CADASIL 1 |
Gt(ROSA)26Sortm3(NOTCH3*R1031C)Sat/Gt(ROSA)26Sor+ Notch3Gt(PST033)Byg/Notch3Gt(PST033)Byg Tg(Tagln-cre)1Her/0 |
involves: 129 * C57BL/6 * SJL | J:171887 | View | ||||
| CADASIL 1 |
Notch1tm1Grid/Notch1+ Notch3tm1Grid/Notch3tm1Grid |
involves: 129S1/Sv * C57BL/6 | J:227333 | View | ||||
| CADASIL 1 | Notch3hpbk/Notch3hpbk | C57BL/6J-Notch3hpbk/GrsrJ | J:222308 | View | ||||
| CADASIL 1 | Notch3tm1.1Dwr/Notch3+ | involves: 129S/SvEv * Swiss | J:191454 | View | ||||
| CADASIL 1 | Notch3tm1.1Dwr/Notch3tm1.1Dwr | involves: 129S/SvEv * Swiss | J:191454 | View | ||||
| Canavan disease | Aspadeaf14/Aspadeaf14 | BALB/c-Aspadeaf14 | J:211825 | View | ||||
| Canavan disease | Aspanur7/Aspanur7 | involves: C57BL/6J | J:143201, J:226682 | View | ||||
| Canavan disease | Aspatm1a(EUCOMM)Wtsi/Aspatm1a(EUCOMM)Wtsi | involves: C57BL/6J * C57BL/6N | J:172582 | View | ||||
| Canavan disease | Aspatm1Mata/Aspatm1Mata | involves: 129S5/SvEvBrd | J:89100, J:89099 | View | ||||
| hypomyelinating leukodystrophy 2 |
Gjb1tm1Kwi/Y Gjc2tm1(EGFP)Kwi/Gjc2tm1(EGFP)Kwi |
involves: 129P2/OlaHsd * 129S4/SvJae * C57BL/6 | J:219594 | View | ||||
| hypomyelinating leukodystrophy 2 |
Gjc2tm1(EGFP)Kwi/Gjc2tm1(EGFP)Kwi Gjb1tm1Kwi/Gjb1tm1Kwi |
involves: 129P2/OlaHsd * 129S4/SvJae * C57BL/6 | J:219594 | View | ||||
| hypomyelinating leukodystrophy 2 | Gjc2tm2.1Kwi/Gjc2tm2.1Kwi | involves: C57BL/6 * SJL | J:174197 | View | ||||
| hypomyelinating leukodystrophy 6 | Tubb4aem1Avan/Tubb4aem1Avan | involves: C57BL/6 | J:291062 | View | ||||
| hypomyelinating leukodystrophy 6 | Tubb4aJit/Tubb4a+ | FVB.B6-Tubb4aJit | J:321692 | View | ||||
| hypomyelinating leukodystrophy 6 | Tubb4aJit/Tubb4aJit | FVB.B6-Tubb4aJit | J:321692 | View | ||||
| leukoencephalopathy with vanishing white matter |
Eif2b4tm1.1Vdk/Eif2b4+ Eif2b5tm1.1Vdk/Eif2b5tm1.1Vdk |
involves: C57BL/6 * C57BL/6J | J:234659 | View | ||||
| leukoencephalopathy with vanishing white matter |
Eif2b4tm1.1Vdk/Eif2b4tm1.1Vdk Eif2b5tm1.1Vdk/Eif2b5+ |
involves: C57BL/6 * C57BL/6J | J:234659 | View | ||||
| leukoencephalopathy with vanishing white matter |
Eif2b4tm1.1Vdk/Eif2b4tm1.1Vdk Eif2b5tm1.1Vdk/Eif2b5tm1.1Vdk |
involves: C57BL/6 * C57BL/6J | J:234659 | View | ||||
| leukoencephalopathy with vanishing white matter | Eif2b5tm1.1Sidr/Eif2b5tm1.1Sidr | B6(Cg)-Eif2b5tm1.1Sidr | J:278928 | View | ||||
| leukoencephalopathy with vanishing white matter | Eif2b5tm1.1Vdk/Eif2b5tm1.1Vdk | involves: C57BL/6 * C57BL/6J | J:234659 | View | ||||
| leukoencephalopathy with vanishing white matter | Eif2b5tm1Itl/Eif2b5tm1Itl | involves: 129S/SvEv | J:192103 | View | ||||
| leukoencephalopathy with vanishing white matter | Eif2b5tm1Itl/Eif2b5tm1Itl | involves: 129S/SvEv * C57BL * Swiss Webster | J:179801 | View | ||||
| megalencephalic leukoencephalopathy with subcortical cysts 1 | Mlc1tm1.1Vdk/Mlc1tm1.1Vdk | involves: C57BL/6 | J:250520, J:244698 | View | ||||
| normal pressure hydrocephalus | Cfap43em1Sono/Cfap43em1Sono | Not Specified | J:285551 | View | ||||
| Pelizaeus-Merzbacher disease | Dp(XTceal3-Plp1)1Gmh/Y | involves: 129S7/SvEvBrd * C57BL/6 | J:199809 | View | ||||
| Pelizaeus-Merzbacher disease | Plp1jp-msd/Y | involves: BALB/c * C3H * C57BL/6 * C57BL/10 | J:121540 | View | ||||
| Pelizaeus-Merzbacher disease | Plp1jp-rsh/Y | involves: C3H * C57BL/6 * STOCK Rb(1.3)1Bnr | J:121540 | View | ||||
| Pelizaeus-Merzbacher disease | Plp1tm1Frca/Y | B6.129-Plp1tm1Frca | J:143305 | View | ||||
| Pelizaeus-Merzbacher disease | Plp1tm1Kan/? | involves: 129S1/Sv * 129X1/SvJ * C57BL/6 | J:146665 | View | ||||
| Pelizaeus-Merzbacher disease | Tg(Plp)66Kan/0 | B6NCrl.Cg-Tg(Plp)66Kan | J:156106 | View | ||||
| Pelizaeus-Merzbacher disease | Tg(Plp)66Kan/Tg(Plp)66Kan | B6NCrl.Cg-Tg(Plp)66Kan | J:156106 | View | ||||
| Pelizaeus-Merzbacher disease | Tg(Plp)72Kan/Tg(Plp)72Kan | B6N.Cg-Tg(Plp)72Kan | J:229229 | View | ||||
| | Canavan disease | Sod2tm1Cje/Sod2tm1Cje | involves: C57BL/6J | J:45913 | View | |||
| hydrocephalus | Add1tm1Llp/Add1tm1Llp | involves: 129P2/OlaHsd * 129S1/SvImJ * C57BL/6J | J:138919 | View | ||||
| hydrocephalus | Ak7Gt(OST434404)Lex/Ak7Gt(OST434404)Lex | involves: 129S5/SvEvBrd * C57BL/6J | J:185566 | View | ||||
| hydrocephalus | Ak8Gt(OST16378)Lex/Ak8Gt(OST16378)Lex | involves: 129S5/SvEvBrd * C57BL/6J | J:185566 | View | ||||
| hydrocephalus | Ccdc39prh/Ccdc39prh | involves: A/J * FVB/N | J:253968 | View | ||||
| hydrocephalus | Ccdc85chhy/Ccdc85chhy | involves: BALB/cHeA * STS/A | J:93608 | View | ||||
| hydrocephalus | Celsr2tm1Lex/Celsr2tm1Lex | involves: 129S5/SvEvBrd * C57BL/6J | J:185566 | View | ||||
| hydrocephalus | Dpcd/PollGt(OST280355)Lex/Dpcd/PollGt(OST280355)Lex | involves: 129S5/SvEvBrd * C57BL/6Brd | J:185566 | View | ||||
| hydrocephalus | E2f5tm1Dli/E2f5tm1Dli | either: (involves: 129S1/Sv * BALB/c) or (involves: 129S1/Sv * C57BL/6) | J:47283 | View | ||||
| hydrocephalus | Hydinhy3/Hydinhy3 | involves: CBA | J:5426 | View | ||||
| hydrocephalus | Kif27Gt(OST441915)Lex/Kif27Gt(OST441915)Lex | involves: 129S5/SvEvBrd * C57BL/6J | J:185566 | View | ||||
| hydrocephalus | Mboat7tm1Lex/Mboat7tm1Lex | involves: 129S5/SvEvBrd * C57BL/6J | J:185566 | View | ||||
| hydrocephalus | Nme5tm1Lex/Nme5tm1Lex | involves: 129S5/SvEvBrd * C57BL/6J | J:185566 | View | ||||
| hydrocephalus | Nme7Gt(OST31116)Lex/Nme7Gt(OST31116)Lex | involves: 129S5/SvEvBrd * C57BL/6Brd | J:185566 | View | ||||
| hydrocephalus | PomkGt(OST243203)Lex/PomkGt(OST243203)Lex | involves: 129S5/SvEvBrd * C57BL/6J | J:185566 | View | ||||
| hydrocephalus | Stk36tm1Lex/Stk36tm1Lex | involves: 129S5/SvEvBrd * C57BL/6J | J:185566 | View | ||||
| hydrocephalus |
Tg(Sox3)1Pqt/0 Tg(Sox3-EGFP)NrPqt/0 |
involves: C57BL/6 * CBA | J:184232 | View | ||||
| hydrocephalus | Ulk4tm1Lex/Ulk4tm1Lex | involves: 129S5/SvEvBrd * C57BL/6J | J:185566 | View | ||||
| leukodystrophy | Clcn2nmf289/Clcn2nmf289 | CByJ.Cg-Clcn2nmf289/Pjn | J:243745 | View | ||||
| leukoencephalopathy with vanishing white matter | Eif2b4tm1.1Vdk/Eif2b4tm1.1Vdk | involves: C57BL/6 * C57BL/6J | J:234659 | View | ||||
| megalencephalic leukoencephalopathy with subcortical cysts | Hepacamtm1Vlcg/Hepacamtm1Vlcg | Not Specified | J:244698 | View | ||||
| neuroaxonal dystrophy | Borcs7Q87X/Borcs7Q87X | involves: 129S6/SvEvTac * C57BL/6 * DBA/2 | J:271219 | View | ||||
| normal pressure hydrocephalus | Ak9em1Mdj/Ak9em1Mdj | C57BL/6-Ak9em1Mdj | J:344356 | View | ||||
| normal pressure hydrocephalus | Cwh43em1Mdj/Cwh43+ | C57BL/6-Cwh43em1Mdj | J:310176 | View | ||||
| normal pressure hydrocephalus | Cwh43em1Mdj/Cwh43em1Mdj | C57BL/6-Cwh43em1Mdj | J:310176 | View | ||||
| normal pressure hydrocephalus | Cwh43em1Mdj/Cwh43em2Mdj | C57BL/6-Cwh43em1Mdj/Cwh43em2Mdj | J:310176 | View | ||||
| normal pressure hydrocephalus | p23-ST1/p23-ST1 | C57BL/6J-p23-ST1 | J:158278 | View | ||||
| obstructive hydrocephalus | Myo9atm1.2Bah/Myo9atm1.2Bah | involves: C57BL/6 * SJL | J:251454 | View | ||||
| Transgenes and Other Mutations | adult-onset autosomal dominant demyelinating leukodystrophy | Tg(Plp1-LMNB1)1108Qsp/0 | involves: FVB/N | J:226169 | View | |||
| adult-onset autosomal dominant demyelinating leukodystrophy | Tg(Plp1-Lmnb1)#Yfu/0 | involves: FVB | J:197168 | View | ||||
| Alexander disease | Tg(GFAP)7Mes/0 | involves: FVB/N | J:99256 | View | ||||
| Alexander disease | Tg(GFAP)10Mes/0 | involves: FVB/N | J:99256 | View | ||||
| Alexander disease | Tg(Gfap-GFAP*R239H)60TMIke/0 | involves: C57BL/6J | J:139349, J:138318 | View | ||||
| CADASIL 1 | Tg(Notch3*R169C)88Bbb/0 | involves: FVB/N | J:220006 | View | ||||
| leukoencephalopathy with vanishing white matter | Tg(Plp1-Eif2ak3*)18Pop/Tg(Plp1-Eif2ak3*)18Pop | C57BL/6J-Tg(Plp1-Eif2ak3*)18Pop | J:216298 | View | ||||
| Additional Complex Models | CADASIL 1 |
Gt(ROSA)26Sortm3(NOTCH3*R1031C)Sat/Gt(ROSA)26Sor+ Tg(Tagln-cre)1Her/0 |
involves: 129S2/SvPas * 129S6/SvEvTac * C57BL/6 * SJL | J:171887 | View | |||
| hydrocephalus | Fzd3tm1(FZD3)Lex/Fzd3tm1(FZD3)Lex | involves: 129S5/SvEvBrd * C57BL/6J | J:185566 | View | ||||
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No similarity to the expected human disease phenotype was found.
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| Disease Term | Allelic Composition | Genetic Background | Reference | Phenotypes | ||||
| NOT Models | adrenoleukodystrophy | Acsbg1tm1Mld/Acsbg1tm1Mld | involves: 129S1/Sv * 129X1/SvJ * C57BL/6 | J:150391 | View | |||
| CADASIL 1 | Notch3tm1Grid/Notch3tm1Grid | involves: 129S1/Sv * C57BL/6 | J:227333 | View | ||||
| CADASIL 1 | Notch3tm1Ul/Notch3+ | Not Specified | J:95927 | View | ||||
| CADASIL 1 | Notch3tm1Ul/Notch3tm1Ul | Not Specified | J:95927 | View | ||||
| hypomyelinating leukodystrophy 7 | Polr3aGt(LG04)Osb/Polr3atm1Bebr | involves: C57BL/6 * C57BL/6J * DBA/2 | J:250577 | View | ||||
| hypomyelinating leukodystrophy 7 | Polr3atm1.1Bebr/Polr3atm1.1Bebr | involves: C57BL/6J | J:250577 | View | ||||