Phenotypes Associated with This Genotype

Genotype
MGI:5450673

• Allelic Composition: Tg(Thy1-MAPT*P301S)2541Godt/Tg(Thy1-MAPT*P301S)2541Godt
• Genetic Background: involves: C57BL/6J * CBA/Ca

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

behavior/neurological

limb grasping ( J:108870 )

• mice are unable to extend their hindlimbs when lifted by the tail

tremors ( J:108870 )

• mice develop tremor at 5-6 months of age

paraparesis ( J:108870 )

• mice develop severe paraparesis by 5-6 months of age

muscle

skeletal muscle fiber atrophy ( J:108870 )

• atrophic, angulated muscle fibers are frequently seen in hindlimb skeletal muscle, indicative of denervation atrophy

muscle weakness ( J:108870 )

• mice develop general muscle weakness at 5-6 months of age

nervous system

tau protein deposits ( J:108870 )

• large numbers of abnormal tau filaments are seen in the cytoplasm and processes of nerve cells from 5-6 month old mice

• majority of filaments resemble half-twisted ribbons seen in FTDP-17, while a minority resemble paired helical filaments of Alzheimers disease

abnormal glial cell morphology ( J:108870 )

astrocytosis ( J:108870 )

• reactive astrocytosis in the motor neurons of the anterior horn of the spinal cord

decreased motor neuron number ( J:108870 )

• reduction in the number of motor neurons in the ventral gray matter of 6 month old mutants

• 49% reduction in the number of motor neurons in the anterior horn of the spinal cord

neurodegeneration ( J:108870 )

• neuronal cell bodies are often swollen or severely atrophic

• occasionally pyknotic neurons are surrounded by glial cells, suggesting neuronophagia

vision/eye

eye inflammation ( J:108870 )

immune system

eye inflammation ( J:108870 )

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
frontotemporal dementia		DOID:9255	OMIM:600274	J:108870