Mouse Genome Informatics
tg1
    Tg(Thy1-TARDBP)6Singh/Tg(Thy1-TARDBP)6Singh
involves: C57BL/6 * C57BL/6J * SJL
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
mortality/aging
• average survival of 6.7 months

behavior/neurological
• develops abnormal limb reflex at 2 months
• shows up to 6 fold reduced motor performance at about 4 months of age

nervous system
• increase level of activated caspase-3 and cleaved poly(ADP-ribose) polymerase (PARP)
• decreased number of neurons in the lumbosacral region.
• highly transgene dose dependent
• large neuronal cytoplasmic and intranuclear inclusions are present to some extent in hippocampal/subicular neurons
• neuronal loss is seen in all affected brain regions
• large neuronal cytoplasmic and intranuclear inclusions are present to some extent in hippocampal/subicular neurons
• neuronal loss is seen in all affected brain regions
• large neuronal cytoplasmic and intranuclear inclusions are present in cortical layer V of the anterior cortex including the primary motor cortex
• neuronal loss is seen in all affected brain regions including both the superficial and deep cortical layers of the anterior cortex
• large neuronal cytoplasmic and intranuclear inclusions are present in somatosensory areas of the hind- and forelimbs
• neuronal loss is seen in all affected brain regions
• highly transgene dose dependent
• neuronal loss is seen in all affected brain regions including both the superficial and deep cortical layers of the anterior cortex
• present in cortical layer V of the anterior cortex including the primary motor cortex and somatosensory areas of the hind- and forelimbs and to some extent in the hippocampal/subicular neurons
• number of neurons in the lumbosacral region is significantly lower
• quantitative neuronal loss is shown in motor cortex at 6 months
• number of neurons in the lumbosacral region of the spinal cord is significantly lower
• atrophy and increased number of pyknotic neurons in the ventral horn region of the lumbosacral and cervical spinal cord occurs in a transgene dose dependent manner

hematopoietic system
• highly transgene dose dependent

immune system
• highly transgene dose dependent

cellular
• increase level of activated caspase-3 and cleaved poly(ADP-ribose) polymerase (PARP)
• decreased number of neurons in the lumbosacral region.