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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 (J:157550)
• average survival time is 24 days (J:157550)

behavior/neurological
• at about 14 days of age mice develop hindlimb grasping (J:157550)
• at about 14 days of age mice develop hindlimb grasping (J:157550)
• show a statistically significant about 2.5 fold reduced performance on rotarod (J:157550)
• show a statistically significant about 2.5 fold reduced performance on rotarod (J:157550)
• wide based stance (J:157550)
• wide based stance (J:157550)
• wide based stance, small stride, and frequent off line stumbling (J:157550)
• wide based stance, small stride, and frequent off line stumbling (J:157550)
• footprint analysis shows a significant about 2 fold decrease in the stride of hindlimbs and of forelimbs (J:157550)
• footprint analysis shows a significant about 2 fold decrease in the stride of hindlimbs and of forelimbs (J:157550)
• after about 22 days of age, an extremely rapid disease progression begins with mice becoming completely paralyzed and dying within 3-4 days (J:157550)
• after about 22 days of age, an extremely rapid disease progression begins with mice becoming completely paralyzed and dying within 3-4 days (J:157550)

muscle
• at about 22 days of age, fasciculations and spasms of facial muscles are observed (J:157550)
• at about 22 days of age, fasciculations and spasms of facial muscles are observed (J:157550)

nervous system
• highly transgene dose dependent (J:157550)
• highly transgene dose dependent (J:157550)
• large neuronal cytoplasmic and intranuclear inclusions are present to some extent in hippocampal/subicular neurons (J:157550)
• neuronal loss is seen in all affected brain regions (J:157550)
• large neuronal cytoplasmic and intranuclear inclusions are present to some extent in hippocampal/subicular neurons (J:157550)
• neuronal loss is seen in all affected brain regions (J:157550)
• loss of CA3 hippocampal neurons and degeneration of Purkinje cells (J:157550)
• loss of CA3 hippocampal neurons and degeneration of Purkinje cells (J:157550)
• large neuronal cytoplasmic and intranuclear inclusions are present to some extent in hippocampal/subicular neurons (J:157550)
• neuronal loss is seen in all affected brain regions (J:157550)
• large neuronal cytoplasmic and intranuclear inclusions are present to some extent in hippocampal/subicular neurons (J:157550)
• neuronal loss is seen in all affected brain regions (J:157550)
• large neuronal cytoplasmic and intranuclear inclusions are present in cortical layer V of the anterior cortex including the primary motor cortex (J:157550)
• neuronal loss is seen in all affected brain regions including both the superficial and deep cortical layers of the anterior cortex (J:157550)
• large neuronal cytoplasmic and intranuclear inclusions are present in cortical layer V of the anterior cortex including the primary motor cortex (J:157550)
• neuronal loss is seen in all affected brain regions including both the superficial and deep cortical layers of the anterior cortex (J:157550)
• large neuronal cytoplasmic and intranuclear inclusions are present in somatosensory areas of the hind- and forelimbs (J:157550)
• neuronal loss is seen in all affected brain regions (J:157550)
• large neuronal cytoplasmic and intranuclear inclusions are present in somatosensory areas of the hind- and forelimbs (J:157550)
• neuronal loss is seen in all affected brain regions (J:157550)
• highly transgene dose dependent (J:157550)
• highly transgene dose dependent (J:157550)
• neuronal loss is seen in all affected brain regions including both the superficial and deep cortical layers of the anterior cortex (J:157550)
• large neuronal cytoplasmic and intranuclear inclusions are present in somatosensory areas of the hind- and forelimbs (J:157550)
• neuronal loss is seen in all affected brain regions including both the superficial and deep cortical layers of the anterior cortex (J:157550)
• large neuronal cytoplasmic and intranuclear inclusions are present in somatosensory areas of the hind- and forelimbs (J:157550)
• 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 (J:157550)
• 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 (J:157550)
• number of neurons in the lumbosacral region is significantly lower (J:157550)
• number of neurons in the lumbosacral region is significantly lower (J:157550)
• quantitative neuronal loss is shown in motor cortex at 24 days (J:157550)
• number of neurons in the lumbosacral region of the spinal cord is significantly lower (J:157550)
• quantitative neuronal loss is shown in motor cortex at 24 days (J:157550)
• number of neurons in the lumbosacral region of the spinal cord is significantly lower (J:157550)
• vacuolar degeneration of several cranial motor nuclei is observed (J:157550)
• vacuolar degeneration of several cranial motor nuclei is observed (J:157550)
• 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 (J:157550)
• 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 (J:157550)

hematopoietic system
• highly transgene dose dependent (J:157550)
• highly transgene dose dependent (J:157550)

immune system
• highly transgene dose dependent (J:157550)
• highly transgene dose dependent (J:157550)


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
02/02/2016
MGI 6.02
The Jackson Laboratory