Phenotypes associated with this allele
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Thy1-FUS*)1Dit mutation
(0 available)
Tg(Thy1-TARDBP)4Singh mutation
(1 available)
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mortality/aging
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• shorter life span, with mice starting to die around 20 weeks of age and 50% survival at around 40 weeks of age
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behavior/neurological
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• mice develop an abnormal hindlimb reflex, characterized by hindlimb retraction when lifted by the tail from 8 weeks of age
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• mice show unstable walking and tremor-like movements that become increasing severe with age
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• mice show progressive motor impairment on the accelerating rotarod which is prominent by 12 weeks of age
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• mice show progressive motor weakening on the hanging wire test at about 12 weeks of age
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• mice show abnormal gait, with irregularly spaced shorter strides and uneven left-right step pattern at 1 year of age
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• mice show irregularly spaced shorter strides at 1 year of age
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hematopoietic system
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• microgliosis in the frontal cortex
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immune system
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• microgliosis in the frontal cortex
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nervous system
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• microgliosis in the frontal cortex
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• reactive astrocytosis in the frontal cortex
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• the number of nuclear bodies known as Gemini of coiled bodies in cortical neurons of motor cortex is reduced
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Thy1-TARDBP)4Singh mutation
(1 available)
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mortality/aging
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• average survival time is 24 days
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behavior/neurological
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• at about 14 days of age mice develop hindlimb grasping
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• show a statistically significant about 2.5 fold reduced performance on rotarod
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• wide based stance, small stride, and frequent off line stumbling
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• footprint analysis shows a significant about 2 fold decrease in the stride of hindlimbs and of forelimbs
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• after about 22 days of age, an extremely rapid disease progression begins with mice becoming completely paralyzed and dying within 3-4 days
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muscle
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• at about 22 days of age, spasms of facial muscles are observed
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• at about 22 days of age, fasciculations of facial muscles are observed
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nervous system
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• highly transgene dose dependent
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• large neuronal cytoplasmic and intranuclear inclusions are present to some extent in hippocampal/subicular neurons
• neuronal loss is seen in all affected brain regions
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• loss of CA3 hippocampal neurons and degeneration of Purkinje cells
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• large neuronal cytoplasmic and intranuclear inclusions are present to some extent in hippocampal/subicular neurons
• neuronal loss is seen in all affected brain regions
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• 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
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• 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
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• highly transgene dose dependent
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• 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
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• 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
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• number of neurons in the lumbosacral region is significantly lower
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• 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
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• vacuolar degeneration of several cranial motor nuclei is observed
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• 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
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hematopoietic system
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• highly transgene dose dependent
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immune system
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• highly transgene dose dependent
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Thy1-TARDBP)4Singh mutation
(1 available)
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behavior/neurological
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• abnormal limb reflex at about 14 months of age
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• 40% reduced motor performance at about 15 months of age
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nervous system
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• highly transgene dose dependent
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• large neuronal cytoplasmic and intranuclear inclusions are present to some extent in hippocampal/subicular neurons
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• large neuronal cytoplasmic and intranuclear inclusions are present to some extent in hippocampal/subicular neurons
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• large neuronal cytoplasmic and intranuclear inclusions are present in cortical layer V of the anterior cortex including the primary motor cortex
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• large neuronal cytoplasmic and intranuclear inclusions are present in somatosensory areas of the hind- and forelimbs
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• highly transgene dose dependent
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• 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
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• 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
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hematopoietic system
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• highly transgene dose dependent
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immune system
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• highly transgene dose dependent
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Analysis Tools