Phenotypes Associated with This Genotype

Genotype
MGI:5575509

• Allelic Composition: Tg(Prnp-SNAP25/HTT*150Q)8Xjl/0
• Genetic Background: involves: FVB

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

Appearance of old Tg(Prnp-SNAP25/HTT*150Q)8Xjl/0 (150Q) mouse and control littermate at 13 months of age

mortality/aging

premature death ( J:201806 )

• mice die between 4 and 15 months of age

growth/size/body

weight loss ( J:201806 )

• weight loss is seen after around 9 months of age

behavior/neurological

increased anxiety-related response ( J:201806 )

• in the light-dark box test, mice show a reduction in the time they stay in the light box with a decrease in cross time, suggesting increased anxiety

decreased startle reflex ( J:201806 )

• mice show decreased acoustic startle responses

limb grasping ( J:201806 )

impaired coordination ( J:201806 )

• mice show motor function deficits on the rotarod when they reach 11 months of age

• in the beam-walking assay, mice show a higher number of slips on the beam than controls, although the time to traverse the beam is no different

nervous system

astrocytosis ( J:201806 )

• increase in astrocytic gliosis in the cortex, striatum, and cerebellum

reduced long-term potentiation ( J:201806 )

• long-term potentiation (LTP) of field excitatory postsynaptic potentials (fEPSPs) is lower in the sensorimotor cortex

decreased synaptic glutamate release ( J:201806 )

• glutamate release is selectively reduced in the striatum

decreased paired-pulse facilitation ( J:201806 )

• the amplitude of paired-pulse facilitation (PPF) induced by <100 ms intervals is lower than in wild-type mice

skeleton

kyphosis ( J:201806 )

• hunchback appearance

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
Huntington's disease		DOID:12858	OMIM:143100	J:201806