Phenotypes Associated with This Genotype

Genotype
MGI:6286248

• Allelic Composition: Tg(Prnp-MAPT*P301S)50Hiw/0
• Genetic Background: B6.Cg-Tg(Prnp-MAPT*P301S)50Hiw

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

mortality/aging

premature death ( J:213361 )

♂ • life expectancy is reduced

growth/size/body

decreased body weight ( J:213361 )

♂ • lower mean body weight, with this difference increasing with age

behavior/neurological

impaired spatial working memory ( J:213361 )

♂ • in the Y-maze test of short-term spatial memory, spontaneous alternation behavior is impaired in 5 month old mice

abnormal response to novel object ( J:213361 )

♂ • in the novel object recognition test, 5 month old mutant mice show less exploratory preference for the novel object than for the previously presented object

♂ • in the novel object recognition test, 5 month old mutant mice exhibit similar exploratory activity around both trial objects as wild-type mice although mutants tend to explore objects for longer period than wild-type mice

abnormal motor capabilities/coordination/movement ( J:213361 )

♂ • mice exhibit motor deficits at 8.5 months of age but not at 5 months

limb grasping ( J:213361 )

♂ • mice cross their hindlimbs when lifted by the tail

nervous system

tau protein deposits ( J:213361 )

♂ • mice exhibit age dependent intracellular accumulation and insolubilzation of tau by 9 months of age, indicating age-dependent tauopathy-like tau accumulation

abnormal axonal transport ( J:213361 )

♂ • 5 month old mice exhibit impaired axonal transport in the septohippocampal pathway

cellular

abnormal cell physiology ( J:213361 )

♂ • dynamics of tau-associated microtubules are accelerated in both the cortex and hippocampus but not the cerebellum in 4 and 7 month old mice

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
frontotemporal dementia		DOID:9255	OMIM:600274	J:213361
tauopathy		DOID:680		J:213361