Phenotypes Associated with This Genotype

Genotype
MGI:3720701

• Allelic Composition: Tg(Prnp-MAPT*P301L)JNPL3Hlmc/0
• Genetic Background: involves: C57BL/6 * DBA/2 * SJL * SW

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

behavior/neurological

decreased grooming behavior ( J:100965 )

hunched posture ( J:100965 )

paraparesis ( J:100965 )

• progressive hindlimb weakness is observed

decreased vocalization ( J:100965 )

• reduced

nervous system

neurofibrillary tangles ( J:100965 )

• by 3 months of age, neurofibrillary tangles (NFTs) composed of straight fibers sometimes forming complex structures are detected in the spinal cords and pons of transgenic animals, and become more numerous with age; females develop pathology earlier than males

• levels of soluble endogenous and transgenic Tau protein are similar to double transgenic mice

tau protein deposits ( J:100965 )

gliosis ( J:100965 )

• as severe as astrocytosis seen in Tg(APPSWE)2576Kha Tg(Prnp-MAPT*P301L)JNPL3Hlmc double transgenic mice in ventral diencepalon, hindbrain, and spinal cord

abnormal neuron morphology ( J:100965 )

• Tau filaments occupy large proportion of cell volume, displacing nucleus and cytoplasmic organelles and compressing the Golgi apparatus

• dystrophic neurites reactive for APP epitopes are associated with amyloid plaques

neuron degeneration ( J:100965 )

• granulovacuolar degeneration is seen rarely in neurons of amygdala only

vision/eye

abnormal eye physiology ( J:100965 )

• increased eye irritation

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
Alzheimer's disease		DOID:10652		J:100965