Mouse Genome Informatics
tg1
    Tg(TARDBP*A315T)#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 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
• 7% increase (an increase of 70 axons) in the number of motor axons with 1-3 um caliber and a 8% decrease (a decrease of 80 axons) in the number of motor axons with 6-9 um caliber in 10 month old mutants
• mice develop ubiquitinated and TARDBP+ inclusions in the spinal cord, cortex, and hippocampus by 10 months of age
• abnormal accumulation of neurofilament and peripherin proteins in inclusions in large motor neurons and the brain at 10 months of age

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