Phenotypes associated with this allele

• Allele Symbol: Tg(YAC128)55Hay
• Allele Name: transgene insertion 55, Michael Hayden
• Allele ID: MGI:5432147
• Summary: 2 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
tg1	Tg(YAC128)55Hay/Tg(YAC128)55Hay	FVB/N-Tg(YAC128)55Hay	MGI:5432190
tg2	Tg(YAC128)55Hay/0	FVB/N-Tg(YAC128)55Hay	MGI:5432192

Genotype
MGI:5432190

tg1

• Allelic Composition: Tg(YAC128)55Hay/Tg(YAC128)55Hay
• Genetic Background: FVB/N-Tg(YAC128)55Hay

• ♀: phenotype observed in females
• ♂: phenotype observed in males
• N: normal phenotype

nervous system

increased neuron apoptosis ( J:105728 )

• general increase in stress-induced apoptosis in primary cultures of striatal neurons

• in cultured striatal medium spiny neurons in response to mitochondria stress

increased susceptibility to neuronal excitotoxicity ( J:105728 , 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)

• in medium spiny neurons in response to NMDA (500 um) exposure compared to wild-type and mice hemizygous for this transgene (J:105723)

abnormal striatum morphology ( J:105723 )

• decrease in striatal volume at 12 months of age

neuronal intranuclear inclusions ( J:105723 )

• at 18 months of age in the striatum and cortex

behavior/neurological

impaired coordination ( J:105723 )

• 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

decreased locomotor activity ( J:105723 )

• approaches statistical significance at 12 months of age

cellular

increased neuron apoptosis ( J:105728 )

• general increase in stress-induced apoptosis in primary cultures of striatal neurons

• in cultured striatal medium spiny neurons in response to mitochondria stress

increased susceptibility to neuronal excitotoxicity ( J:105728 , 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)

• in medium spiny neurons in response to NMDA (500 um) exposure compared to wild-type and mice hemizygous for this transgene (J:105723)

homeostasis/metabolism

increased susceptibility to neuronal excitotoxicity ( J:105728 , 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)

• in medium spiny neurons in response to NMDA (500 um) exposure compared to wild-type and mice hemizygous for this transgene (J:105723)

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
Huntington's disease		DOID:12858	OMIM:143100	J:105723 , J:105728

Genotype
MGI:5432192

tg2

• Allelic Composition: Tg(YAC128)55Hay/0
• Genetic Background: FVB/N-Tg(YAC128)55Hay

nervous system

nervous system phenotype ( J:105723 )

N • no neuronal inclusions are detected at 18 months of age in the cortex or striatum

increased susceptibility to neuronal excitotoxicity ( J:105723 )

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

abnormal striatum morphology ( J:105723 )

• decrease in striatal volume at 12 months of age

• volume is increased compared to mice hemizygous for Tg(YAC128)53Hay

behavior/neurological

impaired coordination ( J:105723 )

• late onset, beginning at 9 months of age

• disease progression is slower than in mice hemizygous for Tg(YAC128)53Hay or homozygous mice

cellular

increased susceptibility to neuronal excitotoxicity ( J:105723 )

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

homeostasis/metabolism

increased susceptibility to neuronal excitotoxicity ( J:105723 )

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

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
Huntington's disease		DOID:12858	OMIM:143100	J:105723