A simple composite phenotype scoring system for evaluating mouse models of cerebellar ataxia

SJ Guyenet, SA Furrer, VM Damian… - JoVE (Journal of …, 2010 - jove.com
We describe a protocol for the rapid and sensitive quantification of disease severity in
mouse models of cerebella ataxia. It is derived from previously published phenotype
assessments in several disease models, including spinocerebellar ataxias, Huntington s
disease and spinobulbar muscular atrophy. Measures include hind limb clasping, ledge test,
gait and kyphosis. Each measure is recorded on a scale of 0-3, with a combined total of 0-12
for all four measures. The results effectively discriminate between affected and non-affected …