Phenotypes Associated with This Genotype

Genotype
MGI:5703732

• Allelic Composition: Tg(Prnp-MAPT*R406W)21807Dwst/?
• Genetic Background: FVB/N-Tg(Prnp-MAPT*R406W)21807Dwst

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

nervous system

microgliosis ( J:95971 )

• prominent microgliosis in the neocortex, amygdala, and hippocampus

neurofibrillary tangles ( J:95971 )

• occasional cell bodies and processes of the pyramidal cells of the hippocampus and amygdala of 14 month old mice show Gallyas-Braak staining, indicating neurofibrillary tangles

tau protein deposits ( J:95971 )

• tau accumulation beginning in the hippocampus and amygdala at 6 months of age, with progressive spreading to the cortices and subcortical areas

• tau localizes predominately in the cell bodies and processes of the neocortical neurons and accumulates in the cell bodies and dendrites of neurons in the anterior horn of the spinal cord and in neuronal processes in the neocortex

• cell bodies of piriform cortex neurons and neurons in the amygdala show massive (15- to 30-nm) straight filaments

• however, no amyloid beta deposition is seen

astrocytosis ( J:95971 )

• astrogliosis is seen in the neocortex, amygdala, and hippocampus at 10 months of age

behavior/neurological

abnormal avoidance learning behavior ( J:95971 )

• 10 month old mice are able to learn to avoid electrical stimuli in the dark compartment within 5 serial trials in 1 week, however the retention time for the avoidance reaction begins to decrease 7 weeks later and worsens over time compared to control mice

impaired coordination ( J:95971 )

• mice show a decrease in retention time on the rotating rod at 10 and 12 months, but not at 5 months, of age

hematopoietic system

microgliosis ( J:95971 )

• prominent microgliosis in the neocortex, amygdala, and hippocampus

immune system

microgliosis ( J:95971 )

• prominent microgliosis in the neocortex, amygdala, and hippocampus

muscle

muscle phenotype ( J:95971 )

N • no atrophy of skeletal muscle is seen

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
frontotemporal dementia		DOID:9255	OMIM:600274	J:95971