Mouse Genome Informatics
tg
    Tg(Thy1-TARDBP)4Singh/0
involves: C57BL/6J * SJL/J
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
behavior/neurological
• abnormal limb reflex at about 14 months of age
• 40% reduced motor performance at about 15 months of age

nervous system
• highly transgene dose dependent
• large neuronal cytoplasmic and intranuclear inclusions are present to some extent in hippocampal/subicular neurons
• large neuronal cytoplasmic and intranuclear inclusions are present to some extent in hippocampal/subicular neurons
• large neuronal cytoplasmic and intranuclear inclusions are present in cortical layer V of the anterior cortex including the primary motor cortex
• large neuronal cytoplasmic and intranuclear inclusions are present in somatosensory areas of the hind- and forelimbs
• highly transgene dose dependent
• 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
• 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

Mouse Models of Human Disease
OMIM IDRef(s)
Amyotrophic Lateral Sclerosis 1; ALS1 105400 J:157550
Frontotemporal Dementia; FTD 600274 J:157550