Research Models
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5 Models
5 Visualizations
ALS-related Research Models
- Sex-specific differences
- Cortical Neuron Loss
- Lower Motor Neuron Loss
- Cytoplasmic Inclusions
- Gliosis
- NMJ Abnormalities
- Muscle Atrophy
- Motor Impairment
- Body Weight
- Premature Death
C9-BAC500 (Brown)
Absent
Not observed.
Absent
Not observed.
Observed
No cytoplasmic mislocalization, or aggregation of TDP-43 in the motor cortex. However, dipeptide repeats accumulated at advanced age and formed small perinuclear inclusion bodies positive for poly-GP.
Absent
No signs of increased activation of microglia or astrocytes in the brain or spinal cord.
Absent
No difference in denervation of neuromuscular junctions at 24 months of age. No difference in motor or sensory spinal nerve root axon number or morphology.
No Data
Muscle histology has not been reported, but no overt muscle atrophy was observed.
Absent
No overt motor deficit as measured by the Rotarod and grip strength.
Absent
Non-significant trend for male C9BAC mice to be heavier than non-Tg controls. Female data have not yet been reported.
Absent
Normal lifespan beyond 2 years in male mice. Female data have not yet been reported.
C9-BACexp (Baloh/Lutz)
Absent
Not observed.
Absent
Not observed.
Observed
RNA foci throughout the nervous system starting at 3 months of age. Foci comprised of RNA transcripts in both the sense and antisense directions. Age-associated formation of dipeptide aggregates, e.g., poly-GP.
Absent
No increase in GFAP staining in the brain and spinal cord compared with non-Tg controls, even at 18 months of age.
Absent
Not observed.
Absent
Not observed.
Absent
No abnormalities in grip strength, Rotarod performance, or open-field testing at a young age (3 months) or advanced age (18 months), compared with non-Tg controls.
Absent
No abnormalities in body weight at a young age (3 months) or advanced age (18 months) compared with non-Tg controls.
Absent
Normal lifespan.
C9orf72 Knock-out
Absent
Not observed.
Absent
Not observed.
Absent
Not observed.
Absent
Not observed.
Absent
Not observed.
Absent
Not observed.
Observed
Reduced activity on open-field test. No abnormalities in grip strength or Rotarod performance.
Absent
Not observed.
Absent
Not observed.
C9ORF72(AAV)(G4C2)149
Observed
Cortical neuron loss by 6 months.
No Data
No data.
Observed
Cytoplasmic inclusions containing dipeptide repeat proteins (DPRs) derived from sense RNA seen in the cortex, hippocampus, cerebellum, and spinal cord; inclusions containing DPRs produced from antisense transcripts seen in the cortex and occasionally in the hippocampus.
Observed
Astrogliosis in the cortex by 3 months.
No Data
No data.
No Data
No data.
Observed
Deficits in the hanging wire test emerge between 3 and 6 months.
No Data
No data.
No Data
No data.
C9ORF72(AAV)(G4C2)66
Observed
Compared with mice expressing 2-repeats, the 66-repeat mice had 17 percent fewer neurons in the cortex at 6 months of age and 11 percent fewer Purkinje cells in the cerebellum. At this age neurons in the hippocampus and thalamus were not affected.
Absent
At 6 months, neuronal loss in the spinal cord was not detected.
Observed
By 6 months, inclusions of C9RAN dipeptides were present in neurons of the cortex and hippocampus, and to a lesser extent in the cerebellum and spinal cord. Inclusions contained polyGA, polyGP, and polyGR dipeptides and were largely ubiquitin-positive.
Observed
Astrogliosis in the cortex by 6 months.
No Data
No data.
No Data
No data.
Observed
At 6 months, 66-repeat mice perform as well as 2-repeat mice on the Rotarod on the first day of testing. However, they fail to improve during subsequent trials, suggesting impairments in coordination and/or motor learning.
Observed
At 6 months females had a lower body weight than mice expressing 2-repeats. Body weight did not differ in males.
No Data
No data.