TDP-43 in Mouse Models of ALS and SMA
Author Information
Author(s): Turner Bradley J, Bäumer Dirk, Parkinson Nicholas J, Scaber Jakub, Ansorge Olaf, Talbot Kevin
Primary Institution: University of Oxford
Hypothesis
Does TDP-43 pathology occur in mouse models of ALS and spinal muscular atrophy?
Conclusion
TDP-43 pathology characteristic of human sporadic ALS is not present in mouse models of ALS and SMA.
Supporting Evidence
- TDP-43 mislocalisation was absent in SOD1G93A mice.
- No abnormally phosphorylated TDP-43 species were detected in ALS mouse spinal cord.
- TDP-43 distribution and expression were unchanged in SMA mice.
Takeaway
The study looked at a protein called TDP-43 in mice with ALS and SMA, and found that it didn't behave like it does in humans with ALS.
Methodology
The study used transgenic mouse models and performed immunohistochemistry, immunofluorescence, and biochemical analysis.
Limitations
The study may not capture all aspects of human disease due to the short lifespan of mice.
Participant Demographics
Transgenic SOD1G93A and Smn-/-;SMN2;SMNΔ7 mice were used.
Statistical Information
P-Value
p=0.0004
Statistical Significance
p<0.05
Digital Object Identifier (DOI)
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