Phenotypes Associated with This Genotype

Genotype
MGI:5056394

• Allelic Composition: Tg(Prnp-ATN1)150Dbo/?
• Genetic Background: involves: C3H * C57BL/6

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

mortality/aging

premature death ( J:70362 )

• mice begin dying as early as 10 weeks, many are dead by 50 weeks

behavior/neurological

increased aggression ( J:70362 )

• beginning at 1-2 months

tremors ( J:70362 )

• involuntary tremors beginning at 1-2 months

ataxia ( J:70362 )

• progressive ataxia beginning at 1-2 months

impaired limb coordination ( J:70362 )

• loss of limb coordination beginning at 1-2 months

hyperactivity ( J:70362 )

• beginning at 1-2 months

abnormal mating frequency ( J:70362 )

• decreased breeding efficiency

seizures ( J:70362 )

• seizures are observed in some animals

nervous system

seizures ( J:70362 )

• seizures are observed in some animals

neuronal intranuclear inclusions ( J:70362 )

• inclusion bodies observed in the dentate nucleus of the cerebellum of 12 month old mice

• a few inclusions are observed in the heart

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
dentatorubral-pallidoluysian atrophy		DOID:0060162	OMIM:125370	J:70362