A new video article published by researchers from the ALS Therapy Development Institute in Cambridge, Massachusetts, demonstrates a rapid and simple approach for phenotypic scoring of the SOD1-G93A mouse model of ALS. The mouse model recapitulates several key aspects of the human disease, including motor neuron loss, progressive muscle atrophy and paralysis. However, due to the inherent variability in these transgenic mice, large cohorts are needed in drug efficacy studies in order to reliably assess drug effects (see Working with ALS Mice and Scott et. al., 2008). It is therefore beneficial to have a rapid and objective approach to assess motor phenotype throughout the course of disease. In the September 8 Journal of Visualized Experiments, Theo Hatzipetros and colleagues present a video protocol and accompanying paper on the NeuroScore system to assess hindlimb function and report on representative results throughout disease progression. This can serve as a useful reference for ALS researchers conducting large scale studies in this model.
Reference (subscription required):
Hatzipetros T, Kidd JD, Moreno AJ, Thompson K, Gill A, Vieira FG. A Quick Phenotypic Neurological Scoring System for Evaluating Disease Progression in the SOD1-G93A Mouse Model of ALS. J Vis Exp. 2015 Oct 6;(104). [Pubmed]