Comparison of mouse models for Huntington's disease research

Strain Name Stock Number Developed by: Molecular mutation Phenotype
B6.129S4-Htttm1Mem/J 002688 M. MacDonald KO of exons 4-5 homozygotes die ~ E8
STOCK Htttm2Mem/J 003453 M. MacDonald 20 CAG repeats normal
B6.129-Htttm3Mem/J 003454 M. MacDonald 50 CAG repeats normal
B6.129-Htttm4Mem/J 003597 M. MacDonald 92 CAG repeats nuclear inclusions detected by 12 months
B6.129-Htttm5Mem/J 003598 M. MacDonald 111 CAG repeats nuclear inclusions detected by 10 months
B6.129P2-Htttm2Detl/J 004595 G. Bates 150 CAG repeats nuclear inclusions detected at about 10 months
B6C3-Tg(HD82Gln)81Dbo/J 003627 D. Borchelt 82 CAG repeats Intranuclear inclusions and neuritic aggregates in various regions; animals die at five to six months
FVB/N-Tg(HTT*97Q)IXwy/J 008197 W. Yang 97 CAA-CAG repeats progressive motor impairment by two months of age, brain atrophy by 12 months
B6.129-Gt(ROSA)26Sortm1(HD*103Q)Xwy/J 007708 W. Yang 98 CAG repeats polyQ HD protein is floxed, so expression of neuropathogenic protein is cre-dependent
B6;SJL-Tg(HD)63Aron/J 004360 N. Aronin 100 CAG repeats cortical and striatal neuropathology by five months
CBy.Cg-Tg(HDexon1)61Gpb/J 007578 G. Bates 100 CAG repeats onset of progressive HD phenotype by 15-21 weeks of age
B6CBA-Tg(HDexon1)61Gpb/1J 002809 G. Bates 115 CAG repeats progressive neurological phenotype with later onset than line 62 (below)
B6CBA-Tg(HDexon1)62Gpb/1J 002810 G. Bates 144 CAG repeats R6/2 line exhibits an early-onset (nine to 11 weeks) progressive neurological phenotype including choreiform-like movements, involuntary stereotypic movements, tremor, and epileptic seizures and neuronal intranuclear inclusions which contain both the huntingtin and ubiquitin proteins
FVB/NJ-Tg(YAC72)2511Hay/J 003640 M. Hayden 72 CAG repeats By 12 months of age selective degeneration of medium spiny neurons in the lateral striatum is observed
FVB-Tg(YAC128)53Hay/J 004938 M. Hayden 128 CAG repeats progressive motor impairment appears at six months of age, followed by progressive neurodegeneration, starting at nine months of age, and hypokinesis at 12 months
FVB/N-Tg(YAC353G6)W7Hay/J 007247 M. Hayden 133 CAG repeats contains a mutation conferring resistance to caspase-6 cleavage; serves as control for strain 004938