Optimised and Rapid Pre-clinical Screening in the SOD1G93A Transgenic Mouse Model of Amyotrophic Lateral Sclerosis (ALS)
2011

Rapid Screening in a Mouse Model of ALS

Sample size: 18 publication 10 minutes Evidence: high

Author Information

Author(s): Richard J. Mead, Ellen J. Bennett, Aneurin J. Kennerley, Paul Sharp, Claire Sunyach, Paul Kasher, Jason Berwick, Brigitte Pettmann, Guiseppe Battaglia, Mimoun Azzouz, Andrew Grierson, Pamela J. Shaw

Primary Institution: Sheffield Institute for Translational Neuroscience, University of Sheffield, Sheffield, United Kingdom

Hypothesis

Can a new mouse model of ALS provide more consistent and rapid preclinical screening for therapies?

Conclusion

The new SOD1G93A mouse model shows consistent disease progression and allows for earlier detection of motor function decline, which can improve preclinical testing for ALS therapies.

Supporting Evidence

  • The new mouse model shows a consistent disease course with reduced variability in motor function assessments.
  • Early behavioral changes were detected before visible clinical signs, allowing for earlier intervention.
  • Quantitative measures of motor function were developed to assess early disease progression.

Takeaway

Scientists created a special mouse to study ALS that helps them see problems with movement much earlier, making it easier to test new medicines.

Methodology

The study involved creating a new inbred mouse line and conducting six pharmacology studies to assess motor function and disease progression.

Potential Biases

Potential bias due to the genetic background of the mouse model and the subjective nature of some assessments.

Limitations

The study primarily focuses on a specific mouse model, which may not fully represent human ALS.

Participant Demographics

The study used C57BL/6 SOD1G93A transgenic mice and non-transgenic littermates.

Statistical Information

P-Value

p<0.0001

Statistical Significance

p<0.0001

Digital Object Identifier (DOI)

10.1371/journal.pone.0023244

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