Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disorder with no cure. Of mouse models used to study ALS, the SOD1G93A mouse is the most common, although its use is limited owing to a short lifespan (∼16 weeks). Acevedo-Arozena et al. now characterise in detail the more recently reported SOD1G93Adl low-copy transgenic, which lives for ∼34 weeks. They find that genetic background and gender both influence lifespan and disease course. In addition, SOD1G93Adl mice are abnormal in the startle-response test, which the authors introduce as a new assay for use in SOD1 transgenic mice. The data support wider use of the SOD1G93Adl strain, as it is well suited for studying early-stage ALS. Page 686
Written by editorial staff. © 2011. Published by The Company of Biologists Ltd.
2011
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