Huntington's disease Disease Ontology Browser

Disease Ontology Browser

Huntington's disease (DOID:12858)
Alliance: disease page
Synonyms: HD; Huntington disease; Huntington's chorea
Alt IDs: OMIM:143100, ICD10CM:G10, ICD9CM_2006:333.4, ICD9CM:333.4, KEGG:05016, MESH:D006816, NCI:C82342, UMLS_CUI:C0020179
Definition: A neurodegenerative disease that has_material_basis_in autosomal dominant inheritance and is characterized by unwanted choreatic movements, behavioral and psychiatric disturbances and dementia and has_material_basis_in expansion of CAG triplet repeats (glutamine) resulting in neuron degeneration affecting muscle coordination, cognitive abilities.

Term Browser
Genes (27)
Models (64)

Disease References using Mouse Models (232)

Mouse Models

Human Disease Modeled: Huntington's disease
Associated Mouse Gene: Htt

Allelic Composition	Genetic Background	Reference	Phenotypes
Htttm1Mfc/Htttm1Mfc	involves: 129S1/Sv * C57BL/6	J:85298, J:141673	View
Htttm1Detl/Htttm1Detl	involves: 129P2/OlaHsd * C57BL/6J	J:67074	View
Htttm1.1Pfs/Htt+	involves: 129S4/SvJae * C57BL/6	J:54906	View
Htttm2.1Pfs/Htt+	involves: 129X1/SvJ * C57BL/6	J:54906	View
Htttm2Msl/Htttm2Msl	involves: 129S1/Sv * C57BL/6J	J:76018, J:79137	View
Htttm1.1Tna/Htttm1.1Tna	involves: 129P2/OlaHsd * C57BL/6J	J:76017	View
Htttm1Detl/Htt+	involves: 129P2/OlaHsd * C57BL/6J	J:67074	View
Htttm1Hay/Htt+	involves: C57BL/6J	J:25941	View
Htttm1Szi/Htttm2Szi Tg(Camk2a-cre)2Szi/0	involves: 129S/SvEv * 129S1/Sv * C57BL/6 * CBA	J:65520	View
Htttm4Mem/Htttm4Mem	involves: 129S1/Sv * 129X1/SvJ * CD-1	J:60937, J:99425, J:111237	View
Htttm1.1Tna/Htt+	involves: 129P2/OlaHsd * C57BL/6J	J:76017	View
Htttm5Detl/Htt+	involves: C57BL/6	J:250587	View
Tg(Camk2a-tTA)1Mmay/0 Tg(tetO-HTT*94Q,-lacZ)1Rhn/0	involves: C57BL/6 * CBA	J:61490	View
Htttm1Szi/Htttm2Szi Tg(Camk2a-cre)1Szi/0	involves: 129S/SvEv * 129S1/Sv * C57BL/6 * CBA	J:65520	View
Htttm1Hay/Htttm1Hay Tg(HTT*97Q)IXwy/0 Tg(YAC18)18Hay/Tg(YAC18)18Hay	FVB.Cg-Htt^tm1Hay Tg(HTT*97Q)IXwy Tg(YAC18)18Hay	J:191147, J:215223	View
Htttm2Detl/Htttm2Detl	involves: 129P2/OlaHsd * C57BL/6J	J:123681	View
Htttm4Mem/Htt+	involves: 129S1/Sv * 129X1/SvJ * CD-1	J:60937	View
Htttm5Mem/Htttm5Mem	involves: 129S1/Sv * 129X1/SvJ * CD-1	J:60937, J:99425, J:111237	View
Htttm2Detl/Htt+	B6J.129P2-Htt^tm2Detl	J:220868	View
Htttm5Mem/Htt+	involves: 129S1/Sv * 129X1/SvJ * CD-1	J:60937	View
Htttm1Mem/Htttm6Mem Tg(CAG-cre/Esr1*)5Amc/0	involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * CBA * Swiss Webster	J:260244	View

NOT Models
No similarity to the expected human disease phenotype was found.

Allelic Composition	Genetic Background	Reference	Phenotypes
Htttm1Szi/Htttm1Szi	either: (involves: 129/Sv * C57BL/6J) or (involves: 129/Sv * MF1)	J:29267	View
Htttm1Mem/Htttm1Mem	involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * CD-1	J:27183	View
Htttm1Hay/Htttm1Hay	involves: C57BL/6J	J:25941	View
Htttm7Mem/Htttm7Mem	involves: 129S/SvEv * 129S1/Sv * 129X1/SvJ * CD-1	J:44391	View
Htttm3Mem/Htttm3Mem	involves: 129S1/Sv * 129X1/SvJ * C57BL/6	J:99425	View
Htttm6Mem/Htttm8Mem	involves: 129S1/Sv * 129X1/SvJ * Swiss Webster	J:72915	View
Htttm3Mem/Htttm3Mem	involves: 129S/SvEv * 129S1/Sv * 129X1/SvJ * CD-1	J:44391	View
Htttm2Mem/Htttm2Mem	involves: 129S1/Sv * 129X1/SvJ * CD-1	J:99425	View
Htttm1Mem/Htttm7Mem	involves: 129S/SvEv * 129S1/Sv * 129X1/SvJ * CD-1	J:44391	View

Mouse Models

Human Disease Modeled: Huntington's disease
Associated Mouse Gene: Ppard

Allelic Composition	Genetic Background	Reference	Phenotypes
Tg(CAG-Ppard*E411P)#Als/0 Tg(Nes-cre)1Kln/0	B6J.Cg-Tg(Nes-cre)1Kln Tg(CAG-Ppard*E411P)#Als	J:233176	View

Mouse Models

Human Disease Modeled: Huntington's disease
Associated Mouse Gene: Zdhhc13

Allelic Composition	Genetic Background	Reference	Phenotypes
Zdhhc13Gt(AC0492)Wtsi/Zdhhc13Gt(AC0492)Wtsi	FVB.129P2-Zdhhc13^{Gt(AC0492)Wtsi}	J:191129	View

Mouse Models

Human Disease Modeled: Huntington's disease
Associated Mouse Gene: Zdhhc17

Allelic Composition	Genetic Background	Reference	Phenotypes
Zdhhc17Gt(RRJ233)Byg/Zdhhc17Gt(RRJ233)Byg	FVB.129P2-Zdhhc17^{Gt(RRJ233)Byg}	J:175800	View

Mouse Models

Human Disease Modeled: Huntington's disease

NOT Models
No similarity to the expected human disease phenotype was found.

Allelic Composition	Genetic Background	Reference	Phenotypes
Hap1tm1Xjl/Hap1tm1Xjl	involves: 129S6/SvEvTac * Black Swiss	J:84682	View
Hap1tm1Hay/Hap1tm1Hay	involves: 129S1/Sv * 129X1/SvJ * ICR	J:76174	View

Mouse Models

Human Disease Modeled: Huntington's disease
Associated Mouse Transgene: Tg(CAG-Ppard*E411P)#Als

Allelic Composition	Genetic Background	Reference	Phenotypes
Tg(CAG-Ppard*E411P)#Als/0 Tg(Nes-cre)1Kln/0	B6J.Cg-Tg(Nes-cre)1Kln Tg(CAG-Ppard*E411P)#Als	J:233176	View

Mouse Models

Human Disease Modeled: Huntington's disease
Associated Mouse Transgene: Tg(CMV-HTT*48Q)BTag

Allelic Composition	Genetic Background	Reference	Phenotypes
Tg(CMV-HTT48Q)BTag/Tg(CMV-HTT48Q)BTag	involves: FVB/N	J:50173	View
Tg(CMV-HTT*48Q)BTag/0	involves: FVB/N	J:50173	View

Mouse Models

Human Disease Modeled: Huntington's disease
Associated Mouse Transgene: Tg(CMV-HTT*89Q)ATag

Allelic Composition	Genetic Background	Reference	Phenotypes
Tg(CMV-HTT89Q)ATag/Tg(CMV-HTT89Q)ATag	involves: FVB/N	J:50173	View
Tg(CMV-HTT*89Q)ATag/0	involves: FVB/N	J:50173	View

Mouse Models

Human Disease Modeled: Huntington's disease
Associated Mouse Transgene: Tg(GFAP-HTT*160Q)1Xjl

Allelic Composition	Genetic Background	Reference	Phenotypes
Tg(GFAP-HTT*160Q)1Xjl/0	either: (involves: FVB/NCrl) or (involves: C3H * C57BL/6 * FVB/NCrl)	J:156460	View

Mouse Models

Human Disease Modeled: Huntington's disease
Associated Mouse Transgene: Tg(GFAP-HTT*160Q)31Xjl

Allelic Composition	Genetic Background	Reference	Phenotypes
Tg(GFAP-HTT*160Q)31Xjl/0	either: (involves: FVB/NCrl) or (involves: C3H * C57BL/6 * FVB/NCrl)	J:156460	View

Mouse Models

Human Disease Modeled: Huntington's disease
Associated Mouse Transgene: Tg(HD82Gln)81Gschi

Allelic Composition	Genetic Background	Reference	Phenotypes
Tg(HD82Gln)81Gschi/0	involves: 129S1/Sv * 129X1/SvJ * C3H * C57BL/6 * CD-1	J:172874	View
Tg(HD82Gln)81Gschi/0	involves: C3H/HeJ * C57BL/6	J:53797	View
Cnr1tm1Map/Cnr1tm1Map Tg(HD82Gln)81Gschi/0	involves: 129S1/Sv * 129X1/SvJ * C3H * C57BL/6 * CD-1	J:172874	View

Mouse Models

Human Disease Modeled: Huntington's disease
Associated Mouse Transgene: Tg(HD)63Aron

Allelic Composition	Genetic Background	Reference	Phenotypes
Tg(HD)63Aron/?	involves: C57BL/6 * SJL	J:72772	View

Mouse Models

Human Disease Modeled: Huntington's disease
Associated Mouse Transgene: Tg(HDexon1)61Gpb

Allelic Composition	Genetic Background	Reference	Phenotypes
Tg(HDexon1)61Gpb/?	involves: C57BL/6 * CBA	J:42085, J:203027	View

Mouse Models

Human Disease Modeled: Huntington's disease
Associated Mouse Transgene: Tg(HDexon1)62Gpb

Allelic Composition	Genetic Background	Reference	Phenotypes
Tg(HDexon1)62Gpb/0	B6CBA-Tg(HDexon1)62Gpb/1J	J:99425, J:111237	View
Tg(HDexon1)62Gpb/0	involves: C57BL/6 * CBA	J:42085	View
Tg(HDexon1)62Gpb/0	involves: C57BL/6J * CBA/J	J:36689	View

Mouse Models

Human Disease Modeled: Huntington's disease
Associated Mouse Transgene: Tg(HTT*97Q)IXwy

Allelic Composition	Genetic Background	Reference	Phenotypes
Tg(HTT*97Q)IXwy/0	FVB-Tg(HTT*97Q)IXwy	J:137345	View
Htttm1Hay/Htttm1Hay Tg(HTT*97Q)IXwy/0 Tg(YAC18)18Hay/Tg(YAC18)18Hay	FVB.Cg-Htt^tm1Hay Tg(HTT*97Q)IXwy Tg(YAC18)18Hay	J:191147, J:215223	View

Mouse Models

Human Disease Modeled: Huntington's disease
Associated Mouse Transgene: Tg(HTT*)1Xwy

Allelic Composition	Genetic Background	Reference	Phenotypes
Tg(HTT*)1Xwy/0	FVB-Tg(HTT*)1Xwy	J:157723	View

Mouse Models

Human Disease Modeled: Huntington's disease
Associated Mouse Transgene: Tg(HTT*)LXwy

Allelic Composition	Genetic Background	Reference	Phenotypes
Tg(HTT*)LXwy/0	involves: FVB/NJ	J:219890	View

Mouse Models

Human Disease Modeled: Huntington's disease
Associated Mouse Transgene: Tg(HTT*)NXwy

Allelic Composition	Genetic Background	Reference	Phenotypes
Tg(HTT*)NXwy/0	involves: FVB/NJ	J:219890	View

Mouse Models

Human Disease Modeled: Huntington's disease
Associated Mouse Transgene: Tg(HTT*/EGFP)150Nn

Allelic Composition	Genetic Background	Reference	Phenotypes
Tg(HTT*/EGFP)150Nn/0	involves: C57BL/6 * DBA/2	J:174269	View

Mouse Models

Human Disease Modeled: Huntington's disease
Associated Mouse Transgene: Tg(HTT*/EGFP)190Nn

Allelic Composition	Genetic Background	Reference	Phenotypes
Tg(HTT*/EGFP)190Nn/0	involves: C57BL/6 * DBA/2	J:174269	View

Mouse Models

Human Disease Modeled: Huntington's disease
Associated Mouse Transgene: Tg(Plp1-HTT*150Q)4aXjl

Allelic Composition	Genetic Background	Reference	Phenotypes
Tg(Plp1-HTT*150Q)4aXjl/0	Not Specified	J:221608	View

Mouse Models

Human Disease Modeled: Huntington's disease
Associated Mouse Transgene: Tg(Ppp1r1b-HTT*)1Meeh

Allelic Composition	Genetic Background	Reference	Phenotypes
Tg(Ppp1r1b-HTT*)1Meeh/0	C57BL/6J-Tg(Ppp1r1b-HTT*)1Meeh	J:139807	View

Mouse Models

Human Disease Modeled: Huntington's disease
Associated Mouse Transgene: Tg(Prnp-HTT*82Q)8AGschi

Allelic Composition	Genetic Background	Reference	Phenotypes
Tg(Prnp-HTT*82Q)8AGschi/0	involves: C3H/HeJ * C57BL/6J	J:91795	View

Mouse Models

Human Disease Modeled: Huntington's disease
Associated Mouse Transgene: Tg(Prnp-HTT*82Q)52Caro

Allelic Composition	Genetic Background	Reference	Phenotypes
Tg(Prnp-HTT*82Q)52Caro/0	involves: C3H * C57BL/6	J:179364	View

Mouse Models

Human Disease Modeled: Huntington's disease
Associated Mouse Transgene: Tg(Prnp-HTT*82Q)61Gschi

Allelic Composition	Genetic Background	Reference	Phenotypes
Tg(Prnp-HTT*82Q)61Gschi/0	involves: C3H/HeJ * C57BL/6J	J:91795	View

Mouse Models

Human Disease Modeled: Huntington's disease
Associated Mouse Transgene: Tg(Prnp-HTT*82Q,KRT14-EGFP)C63Dbo

Allelic Composition	Genetic Background	Reference	Phenotypes
Tg(Prnp-HTT*82Q,KRT14-EGFP)C63Dbo/0	Not Specified	J:173756	View

Mouse Models

Human Disease Modeled: Huntington's disease
Associated Mouse Transgene: Tg(Prnp-SNAP25/HTT*150Q)8Xjl

Allelic Composition	Genetic Background	Reference	Phenotypes
Tg(Prnp-SNAP25/HTT*150Q)8Xjl/0	involves: FVB	J:201806	View

Mouse Models

Human Disease Modeled: Huntington's disease
Associated Mouse Transgene: Tg(tetO-HTT*94Q,-lacZ)1Rhn

Allelic Composition	Genetic Background	Reference	Phenotypes
Tg(Camk2a-tTA)1Mmay/0 Tg(tetO-HTT*94Q,-lacZ)1Rhn/0	involves: C57BL/6 * CBA	J:61490	View

Mouse Models

Human Disease Modeled: Huntington's disease
Associated Mouse Transgene: Tg(UBC-GFP/HTT*84Q)22Shya

Allelic Composition	Genetic Background	Reference	Phenotypes
Tg(UBC-GFP/HTT*84Q)22Shya/0	involves: FVB	J:209055	View

Mouse Models

Human Disease Modeled: Huntington's disease
Associated Mouse Transgene: Tg(YAC72)2511Hay

Allelic Composition	Genetic Background	Reference	Phenotypes
Tg(YAC72)2511Hay/Tg(YAC72)2511Hay	FVB/N-Tg(YAC72)2511Hay	J:105728	View
Tg(YAC72)2511Hay/?	involves: FVB/N	J:55405	View

Mouse Models

Human Disease Modeled: Huntington's disease
Associated Mouse Transgene: Tg(YAC128)53Hay

Allelic Composition	Genetic Background	Reference	Phenotypes
Tg(YAC128)53Hay/0	FVB/N-Tg(YAC128)53Hay	J:84453, J:105723, J:111237	View

Mouse Models

Human Disease Modeled: Huntington's disease
Associated Mouse Transgene: Tg(YAC128)55Hay

Allelic Composition	Genetic Background	Reference	Phenotypes
Tg(YAC128)55Hay/Tg(YAC128)55Hay	FVB/N-Tg(YAC128)55Hay	J:105723, J:105728	View
Tg(YAC128)55Hay/0	FVB/N-Tg(YAC128)55Hay	J:105723	View

Mouse Models

Human Disease Modeled: Huntington's disease
Associated Mouse Transgene: Tg(YAC128)#Hay

Allelic Composition	Genetic Background	Reference	Phenotypes
Tg(YAC128)#Hay/0	FVB/N-Tg(YAC128)#Hay	J:98736	View

Mouse Models

Human Disease Modeled: Huntington's disease

Allelic Composition	Genetic Background	Reference	Phenotypes
Drd1tm2Jcd/Drd1+ Tg(Camk2a-cre)2Gsc/0	involves: 129S4/SvJae * FVB/N	J:120070	View

Mouse Models

Human Disease Modeled: Huntington's disease

Allelic Composition	Genetic Background	Reference	Phenotypes
Gt(ROSA)26Sortm1(HD*103Q)Xwy/? Tg(Nes-cre)1Kln/?	involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * SJL	J:99759	View

Mouse Models

Human Disease Modeled: Huntington's disease

Allelic Composition	Genetic Background	Reference	Phenotypes
Gt(ROSA)26Sortm48(HTT)Arte/Gt(ROSA)26Sor+	Not Specified	J:243843	View

Mouse Models

Human Disease Modeled: Huntington's disease

Allelic Composition	Genetic Background	Reference	Phenotypes
Gt(ROSA)26Sortm49(HTT)Arte/Gt(ROSA)26Sor+	Not Specified	J:243843	View

Mouse Models

Human Disease Modeled: Huntington's disease

Allelic Composition	Genetic Background	Reference	Phenotypes
Gt(ROSA)26Sortm50(HTT)Arte/Gt(ROSA)26Sor+	Not Specified	J:243843	View

Mouse Models

Human Disease Modeled: Huntington's disease

Allelic Composition	Genetic Background	Reference	Phenotypes
Gt(ROSA)26Sortm51(HTT)Arte/Gt(ROSA)26Sor+	Not Specified	J:243843	View

Mouse Models

Human Disease Modeled: Huntington's disease

NOT Models
No similarity to the expected human disease phenotype was found.

Allelic Composition	Genetic Background	Reference	Phenotypes
Emx1tm1(cre)Ito/? Gt(ROSA)26Sortm1(HD*103Q)Xwy/?	involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * SJL	J:99759	View

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