Mouse Genome Informatics
tg
    Tg(TARDBP)#Jpj/0
involves: C3H * C57BL/6
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
behavior/neurological
• in the passive avoidance test, mutants exhibit severe cognitive impairments after 7 months of age and especially in the 11th and 13th months
• mice exhibit a learning impairment in the Barnes maze test at 10 months of age, showing a reduction in the time spent in the target quadrant and increased primary errors
• in the probe trial, mutants show a reduction in the time spent in the target quadrant and an increase in the total number of errors
• mice show reduction in latency in the accelerating rotarod tests starting at around 42 weeks of age, indicating age-related motor deficits

nervous system
• age dependent microgliosis and astrogliosis in the brain and spinal cord; mircrogliosis and astrogliosis is less pronounced than in mice expressing the transgene with the G348C mutation
• age dependent microgliosis and astrogliosis in the brain and spinal cord; mircrogliosis and astrogliosis is less pronounced than in mice expressing the transgene with the G348C mutation
• 5% increase (an increase of 50 axons) in the number of motor axons with 1-3 um caliber and a 6% decrease (a decrease of 60 axons) in the number of motor axons with 6-9 um caliber in 10 month old mutants
• 5% of analyzed neuromuscular junctions are denervated in 10 month old mutants, while over 20% are partially denervated

hematopoietic system
• age dependent microgliosis and astrogliosis in the brain and spinal cord; mircrogliosis and astrogliosis is less pronounced than in mice expressing the transgene with the G348C mutation

immune system
• age dependent microgliosis and astrogliosis in the brain and spinal cord; mircrogliosis and astrogliosis is less pronounced than in mice expressing the transgene with the G348C mutation

Mouse Models of Human Disease
OMIM IDRef(s)
Amyotrophic Lateral Sclerosis 1; ALS1 105400 J:195184
Frontotemporal Lobar Degeneration with Tdp43 Inclusions, Grn-Related 607485 J:195184