Phenotypes Associated with This Genotype

Genotype
MGI:5432192

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

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

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