Phenotypes Associated with This Genotype

Genotype
MGI:5700345

• Allelic Composition: Tg(Plp1-HTT*150Q)4aXjl/0
• Genetic Background: Not Specified

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

mortality/aging

premature death ( J:221608 )

• mice start to die at 5 months of age and do not survive past 10 months

growth/size/body

weight loss ( J:221608 )

• body weight begins to decrease at 4 months of age and continues to decrease progressively

behavior/neurological

increased susceptibility to pharmacologically induced seizures ( J:221608 )

• mice show increased sensitivity to flurothyl-induced seizures

abnormal motor capabilities/coordination/movement ( J:221608 )

• 5 month old mice exhibit difficulty walking

limb grasping ( J:221608 )

• 5 month old mice exhibit clasping

tremors ( J:221608 )

• 5 month old mice exhibit tremors

impaired coordination ( J:221608 )

• progressive motor dysfunction on the rotarod with age

decreased locomotor activity ( J:221608 )

• locomotor activity is lower during active times (7 pm to 11 pm)

nervous system

increased susceptibility to pharmacologically induced seizures ( J:221608 )

• mice show increased sensitivity to flurothyl-induced seizures

axon degeneration ( J:221608 )

• degenerated axons, appearing swollen and dark, are seen at 5 months of age, but not at 3 months

demyelination ( J:221608 )

• reduction of myelination in striatum axons is seen at 3 months but not earlier and becomes more severe at 5 months of age

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
Huntington's disease		DOID:12858	OMIM:143100	J:221608