A Simple Composite Phenotype Scoring System for Evaluating Mouse Models of Cerebellar Ataxia
2010

Evaluating Mouse Models of Cerebellar Ataxia

publication Evidence: moderate

Author Information

Author(s): Guyenet Stephan J., Furrer Stephanie A., Damian Vincent M., Baughan Travis D., La Spada Albert R., Garden Gwenn A.

Primary Institution: University of Washington

Hypothesis

The study aims to develop a scoring system to quantify disease severity in mouse models of cerebellar ataxia.

Conclusion

The scoring system effectively discriminates between affected and non-affected mice and quantifies the progression of neurodegenerative disease phenotypes.

Supporting Evidence

  • The scoring system includes measures that are sensitive to disease severity.
  • Each measure is scored on a scale of 0-3, allowing for detailed assessment.
  • The protocol can detect differences in phenotype between strains and over time.

Takeaway

This study created a way to score how sick mice are from a brain disease, helping scientists understand the disease better.

Methodology

The study describes a protocol for scoring disease severity based on four measures: hind limb clasping, ledge test, gait, and kyphosis.

Potential Biases

The experimenter should not know the animal's genotype to prevent bias.

Limitations

Obesity may complicate the interpretation of the results.

Participant Demographics

Transgenic mouse models of spinocerebellar ataxia type 7 (SCA7) were used.

Statistical Information

P-Value

P<0.001

Statistical Significance

p<0.001

Digital Object Identifier (DOI)

10.3791/1787

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