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All mouse models of cerebellar ataxia with phenotypic similarity to the human disease
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| Disease Term | Allelic Composition | Genetic Background | Reference | Phenotypes | ||||
  | ataxia telangiectasia |
Aptxtm1Pmc/Aptxtm1Pmc Atmtm2.1Mfgc/Atmtm2.1Mfgc |
involves: 129 * C57BL/6 | J:316365 | View | |||
| ataxia telangiectasia | Atmtm1.1Mmpl/Atmtm1.1Mmpl | involves: 129S4/SvJaeSor * 129S6/SvEvTac * C57BL/6J | J:226414 | View | ||||
| ataxia telangiectasia | Atmtm1Awb/Atmtm1Awb | either: 129S6/SvEvTac-Atmtm1Awb or (involves: 129S6/SvEvTac * NIH Black Swiss) | J:34193, J:57115 | View | ||||
| ataxia telangiectasia | Atmtm1Bal/Atmtm1Bal | involves: 129S4/SvJae | J:42324, J:44190 | View | ||||
| ataxia telangiectasia | Atmtm1Fwa/Atmtm1Fwa | involves: 129S4/SvJae * C57BL/6 | J:61201 | View | ||||
| ataxia telangiectasia | Atmtm1Led/Atmtm1Led | involves: 129S6/SvEvTac * Black Swiss | J:36561 | View | ||||
| ataxia telangiectasia | Atmtm1Mfl/Atmtm1Mfl | involves: 129T2/SvEms * C57BL/6J | J:69726 | View | ||||
| ataxia telangiectasia | Atmtm1Pmc/Atmtm1Pmc | B6.Cg-Atmtm1Pmc | J:222034 | View | ||||
| ataxia telangiectasia | Atmtm1Pmc/Atmtm1Pmc | involves: 129X1/SvJ * C57BL/6 | J:47752 | View | ||||
| autosomal recessive spinocerebellar ataxia 12 | Wwoxem1Mald/Wwoxem1Mald | FVB/N-Wwoxem1Mald | J:339564 | View | ||||
| autosomal recessive spinocerebellar ataxia 13 | Grm1crv4/Grm1crv4 | BALB/cPas-Grm1crv4 | J:112290 | View | ||||
| autosomal recessive spinocerebellar ataxia 16 | Stub1tm1Cpat/Stub1tm1Cpat | involves: 129S/SvEv * C57BL/6 | J:245069 | View | ||||
| autosomal recessive spinocerebellar ataxia 18 | Grid2ho-15J/Grid2ho-15J | involves: C3HeB/Fe | J:220519 | View | ||||
| autosomal recessive spinocerebellar ataxia 18 | Grid2tm1(cre)Mwa/Grid2tm1(cre)Mwa | involves: C57BL/6N | J:220519 | View | ||||
| Cayman type cerebellar ataxia | Atcayji-hes/Atcayji-hes | C3H/HeJ-Atcayji-hes | J:85793 | View | ||||
| Cayman type cerebellar ataxia | Atcayji/Atcayji | JIGR | J:85793 | View | ||||
| Cayman type cerebellar ataxia | Atcayswd/Atcayswd | Not Specified | J:85793 | View | ||||
| cerebellar ataxia type 42 | Cacna1gem1Ftan/Cacna1g+ | C57BL/6NCrSlc-Cacna1gem1Ftan | J:278129 | View | ||||
| cerebellar ataxia type 42 | Cacna1gem1Ftan/Cacna1gem1Ftan | C57BL/6NCrSlc-Cacna1gem1Ftan | J:278129 | View | ||||
| Charlevoix-Saguenay spastic ataxia | Sacstm1(NCOM)Mfgc/Sacstm1(NCOM)Mfgc | Not Specified | J:182034 | View | ||||
| Charlevoix-Saguenay spastic ataxia | Sacstm2Bebr/Sacstm2Bebr | involves: C57BL/6 | J:217753 | View | ||||
| Friedreich ataxia | Fxnem8Lutzy/Fxnem8Lutzy | C57BL/6J-Fxnem8Lutzy | J:299300 | View | ||||
| Friedreich ataxia | Fxntm1.1Pand/Fxntm1Mkn | B6.Cg-Fxntm1.1Pand Fxntm1Mkn/J | J:242978 | View | ||||
| Friedreich ataxia |
Fxntm1Mkn/Fxntm1Mkn Tg(FXN)YG8Pook/0 |
involves: 129/Sv * C57BL/6 * CBA | J:114840, J:216422 | View | ||||
| Friedreich ataxia |
Fxntm1Mkn/Fxntm1Mkn Tg(FXN)YG22Pook/0 |
involves: 129/Sv * C57BL/6 * CBA | J:114840 | View | ||||
| Friedreich ataxia | Fxntm1Mkn/Fxntm1Pand | involves: 129/Sv * C57BL/6 | J:216422 | View | ||||
| Friedreich ataxia |
Fxntm2Mkn/Fxntm2.1Mkn Tg(Ckmm-cre)1Lrsn/0 |
involves: 129 * C57BL/6J | J:75420 | View | ||||
| Friedreich ataxia |
Fxntm2Mkn/Fxntm2.1Mkn Tg(Eno2-cre)39Jme/0 |
involves: 129 * C57BL/6J | J:75420 | View | ||||
| Friedreich ataxia | Gt(ROSA)26Sortm1(H1/tetO-RNAi:Fxn)Dhg/Gt(ROSA)26Sor+ | B6.129-Gt(ROSA)26Sortm1(H1/tetO-RNAi:Fxn)Dhg | J:254962 | View | ||||
| spinocerebellar ataxia type 1 | Atxn1tm1Hzo/Atxn1+ | involves: 129S7/SvEvBrd * C57BL/6 | J:77225 | View | ||||
| spinocerebellar ataxia type 2 | Atxn2tm2.1Aub/Atxn2tm2.1Aub | B6.129S2-Atxn2tm2.1Aub | J:188118 | View | ||||
| spinocerebellar ataxia type 5 | Sptbn2Gt(XK442)Byg/Sptbn2Gt(XK442)Byg | involves: 129P2/OlaHsd * C57BL/6J | J:158494 | View | ||||
| spinocerebellar ataxia type 5 | Sptbn2tm1Mjac/Sptbn2tm1Mjac | B6.129P2-Sptbn2tm1Mjac | J:159622 | View | ||||
| spinocerebellar ataxia type 6 | Cacna1asmrl/Cacna1asmrl | CXB10/HiAJ-Cacna1asmrl/GrsrJ | J:222308 | View | ||||
| spinocerebellar ataxia type 6 | Cacna1atm2.1Kewa/Cacna1atm2.1Kewa | B6.Cg-Cacna1atm2.1Kewa | J:190729 | View | ||||
| spinocerebellar ataxia type 6 | Cacna1atm3Hzo/Cacna1atm3Hzo | B6.129S7-Cacna1atm3Hzo/J | J:239981 | View | ||||
| spinocerebellar ataxia type 7 | Atxn7tm1Hzo/Atxn7+ | involves: 129S7/SvEvBrd | J:113150, J:179021 | View | ||||
| spinocerebellar ataxia type 7 | Atxn7tm1Hzo/Atxn7+ | involves: 129S7/SvEvBrd * C57BL/6 | J:82072 | View | ||||
| spinocerebellar ataxia type 7 | Atxn7tm1Hzo/Atxn7+ | involves: 129S7/SvEvBrd * C57BL/6J | J:107098 | View | ||||
| spinocerebellar ataxia type 7 | Atxn7tm1Hzo/Atxn7tm1Hzo | involves: 129S7/SvEvBrd | J:179021 | View | ||||
| spinocerebellar ataxia type 7 |
Atxn7tm1Hzo/Atxn7tm1Hzo Kat2atm3.1Roth/Kat2a+ |
involves: 129 * 129S7/SvEvBrd | J:179021 | View | ||||
| spinocerebellar ataxia type 14 | Prkcgem1Jpka/Prkcg+ | FVB/N-Prkcgem1Jpka | J:305637 | View | ||||
| spinocerebellar ataxia type 14 | Prkcgem1Jpka/Prkcgem1Jpka | FVB/N-Prkcgem1Jpka | J:305637 | View | ||||
| spinocerebellar ataxia type 17 |
Tbptm1Xjl/Tbp+ Tg(CAG-cre/Esr1*)5Amc/0 |
involves: 129S/SvEv * C57BL/6 * CBA | J:213011 | View | ||||
| spinocerebellar ataxia type 28 | Afg3l2Emv66/Afg3l2+ | involves: MEV/2Ty | J:151797 | View | ||||
| spinocerebellar ataxia with axonal neuropathy 1 | Tdp1Gt(XD105)Byg/Tdp1Gt(XD105)Byg | involves: 129P2/OlaHsd * C57BL/6 | J:127605 | View | ||||
| | cerebellar ataxia |
Cacna1atm2.1Maag/Cacna1atm2.1Maag Tg(Pcp2-cre)2Mpin/0 |
involves: 129P2/OlaHsd * 129S1/Sv * 129X1/SvJ | J:234599 | View | |||
| Charlevoix-Saguenay spastic ataxia | Ankfy1Gt(RRE069)Byg/Ankfy1+ | B6.129P2-Ankfy1Gt(RRE069)Byg | J:250135 | View | ||||
| spinocerebellar ataxia type 27 | Fgf14tm1Dor/Fgf14tm1Dor | B6.129S6-Fgf14tm1Dor | J:134733 | View | ||||
| | Machado-Joseph disease | Atxn3tm2.1(ATXN3*)Mfig/Atxn3+ | involves: 129 * C57BL/6J | J:218541 | View | |||
| spinocerebellar ataxia type 15 |
Glatm1Kul/Glatm1Kul Itpr1wblo/Itpr1wblo |
B6;129-Glatm1Kul Itpr1wblo/GrsrJ | J:222308 | View | ||||
| spinocerebellar ataxia type 15 |
Glatm1Kul/Y Itpr1wblo/Itpr1wblo |
B6;129-Glatm1Kul Itpr1wblo/GrsrJ | J:222308 | View | ||||
| Transgenes and Other Mutations | dentatorubral-pallidoluysian atrophy | Tg(ATN1*)Q129Stsu/0 | involves: 129S/SvEv * C57BL/6J | J:144379 | View | |||
| dentatorubral-pallidoluysian atrophy | Tg(Eno2-ATN1)3Tx/Tg(Eno2-ATN1)3Tx | B6.Cg-Tg(Eno2-ATN1)3Tx | J:112706 | View | ||||
| dentatorubral-pallidoluysian atrophy | Tg(Eno2-ATN1)14Tx/0 | B6.Cg-Tg(Eno2-ATN1)14Tx | J:112706 | View | ||||
| dentatorubral-pallidoluysian atrophy | Tg(Prnp-ATN1)124Dbo/? | involves: C3H * C57BL/6 | J:70362 | View | ||||
| dentatorubral-pallidoluysian atrophy | Tg(Prnp-ATN1)150Dbo/? | involves: C3H * C57BL/6 | J:70362 | View | ||||
| Machado-Joseph disease | Tg(ATXN3*)67.2Cce/0 | involves: C57BL/6 * CBA | J:76495 | View | ||||
| Machado-Joseph disease |
Tg(ATXN3*)67.2Cce/0 Tg(ATXN3*)84.2Cce/0 |
involves: C57BL/6 * CBA | J:76495 | View | ||||
| Machado-Joseph disease | Tg(ATXN3*)84.2Cce/0 | involves: C57BL/6 * CBA | J:76495 | View | ||||
| Machado-Joseph disease | Tg(ATXN3*)84.2Cce/Tg(ATXN3*)84.2Cce | involves: C57BL/6 * CBA | J:76495 | View | ||||
| Machado-Joseph disease | Tg(CMV-ATXN3*94Q)94Pama/0 | B6.FVB-Tg(CMV-ATXN3*94Q)94Pama | J:163032 | View | ||||
| Machado-Joseph disease | Tg(CMV-ATXN3*94Q)94Pama/Tg(CMV-ATXN3*94Q)94Pama | B6.FVB-Tg(CMV-ATXN3*94Q)94Pama | J:163032 | View | ||||
| Machado-Joseph disease | Tg(CMV-ATXN3*135Q)CPama/0 | C57BL/6-Tg(CMV-ATXN3*135Q)CPama | J:222991 | View | ||||
| Machado-Joseph disease | Tg(Htt-ATXN3*148Q)3746Thsc/0 | involves: C57BL/6N | J:156912 | View | ||||
| Machado-Joseph disease | Tg(Pcp2-ATXN3*69Q)bHirai/0 | Not Specified | J:136101 | View | ||||
| Machado-Joseph disease | Tg(Prnp-ATXN3*70Q)70.61Olri/0 | involves: C57BL/6N | J:122993 | View | ||||
| Machado-Joseph disease | Tg(Prnp-ATXN3*79Q)#Hlw/0 | FVB/N-Tg(Prnp-ATXN3*79Q)#Hlw | J:138381 | View | ||||
| Machado-Joseph disease | Tg(Prnp-ATXN3*148Q)148.19Olri/0 | involves: C57BL/6N | J:122993 | View | ||||
| Machado-Joseph disease | Tg(Prnp-ATXN3*148Q)NLS.28Olri/0 | involves: C57BL/6N | J:122993 | View | ||||
| spinocerebellar ataxia type 1 | Tg(Pcp2-ATXN1*82Q)5Horr/0 | involves: FVB/N | J:166951 | View | ||||
| spinocerebellar ataxia type 1 |
Tg(Pcp2-tTA)3Horr/0 Tg(tetO-ATXN1*82Q)#Horr/Tg(tetO-ATXN1*82Q)#Horr |
involves: FVB/N | J:95453 | View | ||||
| spinocerebellar ataxia type 2 | Tg(ATXN2*72Q)#Plt/0 | FVB/N-Tg(ATXN2*72Q)#Plt | J:224692 | View | ||||
| spinocerebellar ataxia type 2 | Tg(Pcp2-ATXN2*127Q)#Plt/0 | involves: C57BL/6 * DBA/2 | J:191127 | View | ||||
| spinocerebellar ataxia type 5 |
Tg(Pcp2-tTA)3Horr/0 Tg(tetO-SPTBN2*)#Lpwr/0 |
involves: FVB/N | J:215593 | View | ||||
| spinocerebellar ataxia type 7 | Tg(GFAP-ATXN7*92Q)2521Als/0 | involves: C3H/HeJ * C57BL/6J | J:113150 | View | ||||
| spinocerebellar ataxia type 7 | Tg(GFAP-ATXN7*92Q)2542Als/0 | involves: C3H/HeJ * C57BL/6J | J:113150 | View | ||||
| spinocerebellar ataxia type 7 | Tg(Pcp2-SCA7)P7EJman/0 | involves: C57BL/6 * SJL | J:65411 | View | ||||
| spinocerebellar ataxia type 7 | Tg(Prnp-ATXN7*92Q)1963Als/0 | B6J.Cg-Tg(Prnp-ATXN7*92Q)1963Als | J:222402 | View | ||||
| spinocerebellar ataxia type 7 | Tg(Prnp-ATXN7*92Q)6076Als/0 | involves: C3H/HeJ * C57BL/6 | J:71971, J:77530, J:113150 | View | ||||
| spinocerebellar ataxia type 7 | Tg(RHO-SCA7)R7EJman/0 | involves: C57BL/6 * SJL | J:65411 | View | ||||
| spinocerebellar ataxia type 10 | Tg(Prnp-lacZ/ATXN10*)#Teas/0 | involves: FVB/N | J:222081 | View | ||||
| spinocerebellar ataxia type 14 |
Tg(Pcp2-tTA)3Horr/0 Tg(tetO-PRKCG*S361G,-GFP)3Jpka/0 |
involves: FVB/N | J:218354 | View | ||||
| spinocerebellar ataxia type 17 | Tg(Pcp2-TBP*)69Hmhl/0 | involves: FVB/N | J:174239 | View | ||||
| spinocerebellar ataxia type 17 | Tg(Prnp-TBP*)71-16Xjl/0 | FVB/N-Tg(Prnp-TBP*)71-16Xjl | J:130775 | View | ||||
| spinocerebellar ataxia type 17 | Tg(Prnp-TBP*)71-27Xjl/0 | FVB/N-Tg(Prnp-TBP*)71-27Xjl | J:130775 | View | ||||
| spinocerebellar ataxia type 17 | Tg(Prnp-TBP*)105Xjl/0 | FVB/N-Tg(Prnp-TBP*)105Xjl | J:130775 | View | ||||
| Additional Complex Models | Machado-Joseph disease |
Prnptm1Cwe/Prnptm1Cwe Tg(Prnp-tTA)F959Sbp/0 Tg(tetO-ATXN3)2904Olri/Tg(tetO-ATXN3)2904Olri |
involves: 129S7/SvEvBrd * C57BL/6 * FVB | J:154079 | View | |||
| Machado-Joseph disease |
Prnptm1Cwe/Prnptm1Cwe Tg(Prnp-tTA)F959Sbp/Tg(Prnp-tTA)F959Sbp Tg(tetO-ATXN3)2904Olri/0 |
involves: 129S7/SvEvBrd * C57BL/6 * FVB | J:154079 | View | ||||
| Machado-Joseph disease |
Prnptm1Cwe/Prnptm1Cwe Tg(Prnp-tTA)F959Sbp/Tg(Prnp-tTA)F959Sbp Tg(tetO-ATXN3)2904Olri/Tg(tetO-ATXN3)2904Olri |
involves: 129S7/SvEvBrd * C57BL/6 * FVB | J:154079 | 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 | spinocerebellar ataxia type 5 | Sptbn2tm1Mjac/Sptbn2+ | involves: 129P2/OlaHsd | J:163169 | View | |||
| spinocerebellar ataxia type 17 | Tg(Prnp-TBP*)13Xjl/0 | FVB/N-Tg(Prnp-TBP*)13Xjl | J:130775 | View | ||||