Phenotypes Associated with This Genotype

Genotype
MGI:6294293

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

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

mortality/aging

premature death ( J:91795 )

• mice live to 7-9 months of age

growth/size/body

weight loss ( J:91795 )

• mice exhibit reduced weight towards the end of their life

behavior/neurological

poor grooming ( J:91795 )

• mice exhibit poor grooming towards the end of life

limb grasping ( J:91795 )

• mice exhibit hind-limb clasping when suspended by the tail towards the end of life

impaired coordination ( J:91795 )

• by 4.5 months of age, mice show a reduction in performance on the rotarod, with a decrease in latency to fall compared to controls

hunched posture ( J:91795 )

• mice exhibit hunched posture towards the end of life

decreased locomotor activity ( J:91795 )

• 6-7 month old mice are less active than wild-type mice during the dark period

nervous system

neuronal intranuclear inclusions ( J:91795 )

• mice exhibit widespread nuclear inclusions of HTT in most neurons of the CNS, however no cytoplasmic inclusions are detected

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
Huntington's disease		DOID:12858	OMIM:143100	J:91795