Phenotypes Associated with This Genotype

Genotype
MGI:5795743

cx31

• Allelic Composition: Tg(Camk2a-tTA)1Mmay/0; Tg(tetO/Prnp-TARDBP*)2Vle/0
• Genetic Background: involves: C3H/HeJ * C57BL/6J

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

nervous system

astrocytosis ( J:226974 )

• increase in GFAP reactivity 10 months after Dox removal

hippocampal neuron degeneration ( J:226974 )

loss of hippocampal neurons ( J:226974 )

• number of dentate gyrus neurons is decreased in mice at 10, 16 and 19, but not at 5, months after Dox removal as compared to controls

• loss is progressive resulting in an 80% decrease in neurons at 19 months

• reintroduction of Dox clears Tardbp C-terminal fragments (CTF) from the brain and halts neuron loss

neuronal cytoplasmic inclusions ( J:226974 )

• accumulation of ubiquitin and mitochondrial proteins in distal dendrites of the stratum lacunosum moleculare (SLM) layer of the hippocampus 5 months after Dox withdrawal, reintroduction of Dox does not clear ubiquitin

• ubiquitin forms a discrete punctate pattern in SLM

cellular

astrocytosis ( J:226974 )

• increase in GFAP reactivity 10 months after Dox removal