Mouse Genome Informatics
tg1
    Tg(YAC128)55Hay/Tg(YAC128)55Hay
FVB/N-Tg(YAC128)55Hay
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
nervous system
• general increase in stress-induced apoptosis in primary cultures of striatal neurons
• in cultured striatal medium spiny neurons in response to mitochondria stress
• in medium spiny neurons in response to NMDA (500 um) exposure compared to wild-type and mice hemizygous for this transgene (J:105723)
• in cultured striatal medium spiny neurons following exposure to NMDA, the Ca2+ ionophore A23187, or KCl plus the L-type calcium channel agonist FPL 64176 (J:105728)
• apoptosis induced by KCl alone is greater than in mice homozygous for Tg(YAC72)2511Hay (J:105728)
• decrease in striatal volume at 12 months of age
• at 18 months of age in the striatum and cortex

behavior/neurological
• beginning at 4 months of age and becoming worse with age
• disease progression is slower than in mice hemizygous forTg(YAC128)53Hay but faster than in hemizygous mice
• approaches statistical significance at 12 months of age

cellular
• general increase in stress-induced apoptosis in primary cultures of striatal neurons
• in cultured striatal medium spiny neurons in response to mitochondria stress
• in medium spiny neurons in response to NMDA (500 um) exposure compared to wild-type and mice hemizygous for this transgene (J:105723)
• in cultured striatal medium spiny neurons following exposure to NMDA, the Ca2+ ionophore A23187, or KCl plus the L-type calcium channel agonist FPL 64176 (J:105728)
• apoptosis induced by KCl alone is greater than in mice homozygous for Tg(YAC72)2511Hay (J:105728)

homeostasis/metabolism
• in medium spiny neurons in response to NMDA (500 um) exposure compared to wild-type and mice hemizygous for this transgene (J:105723)
• in cultured striatal medium spiny neurons following exposure to NMDA, the Ca2+ ionophore A23187, or KCl plus the L-type calcium channel agonist FPL 64176 (J:105728)
• apoptosis induced by KCl alone is greater than in mice homozygous for Tg(YAC72)2511Hay (J:105728)

Mouse Models of Human Disease
OMIM IDRef(s)
Huntington Disease; HD 143100 J:105723 , J:105728


Mouse Genome Informatics
tg2
    Tg(YAC128)55Hay/0
FVB/N-Tg(YAC128)55Hay
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
nervous system
N
• no neuronal inclusions are detected at 18 months of age in the cortex or striatum (J:105723)
• in medium spiny neurons in response to NMDA (500 um) exposure compared to wild-type
• decrease in striatal volume at 12 months of age
• volume is increased compared to mice hemizygous for Tg(YAC128)53Hay

behavior/neurological
• late onset, beginning at 9 months of age
• disease progression is slower than in mice hemizygous for Tg(YAC128)53Hay or homozygous mice

cellular
• in medium spiny neurons in response to NMDA (500 um) exposure compared to wild-type

homeostasis/metabolism
• in medium spiny neurons in response to NMDA (500 um) exposure compared to wild-type

Mouse Models of Human Disease
OMIM IDRef(s)
Huntington Disease; HD 143100 J:105723