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Phenotypes Associated with This Genotype
Genotype
MGI:4438492
Allelic
Composition
Tg(Thy1-TARDBP)4Singh/Tg(Thy1-TARDBP)4Singh
Genetic
Background
involves: C57BL/6J * SJL/J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Thy1-TARDBP)4Singh mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• average survival time is 24 days

behavior/neurological
• at about 14 days of age mice develop hindlimb grasping
• show a statistically significant about 2.5 fold reduced performance on rotarod
• wide based stance
• wide based stance, small stride, and frequent off line stumbling
• footprint analysis shows a significant about 2 fold decrease in the stride of hindlimbs and of forelimbs
• after about 22 days of age, an extremely rapid disease progression begins with mice becoming completely paralyzed and dying within 3-4 days

muscle
• at about 22 days of age, fasciculations and spasms of facial muscles are observed

nervous system
• 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
• loss of CA3 hippocampal neurons and degeneration of Purkinje cells
• 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
• large neuronal cytoplasmic and intranuclear inclusions are present in somatosensory areas of the hind- and forelimbs
• 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 24 days
• number of neurons in the lumbosacral region of the spinal cord is significantly lower
• vacuolar degeneration of several cranial motor nuclei is observed
• 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


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
11/22/2016
MGI 6.06
The Jackson Laboratory