(A–B) Western blot detecting ATXN1 oligomers (F11G3, top panel) and ATXN1 monomer (11750, middle panel) in the cerebellum of Atxn1154Q/+following immunotherapy (anti-oligomer or control). * in (A) indicated change in exposure of the membrane. (B) Quantification of relative levels of oligomeric and monomeric ATXN1 from (A). ** denotes p<0.01, Student’s T-test. (C) Rotarod assay in all treatment groups over a four-day period (four trials per day, averaged) 6 weeks following onset of immunotherapy (mice 10 weeks of age). n = 12 per genotype; ** denotes p<0.01, ANOVA followed by Tukey’s post hoc test. (D) Kaplan-Meier survival curve shows that animals treated with anti-oligomer immunotherapy (blue line) lived, on average, 3.5 weeks longer than control animals (red line). No death was observed in WT mice receiving immunotherapy (black and grey lines). *** denotes p<0.001, Log-rank (Mantel-Cox) test. n = 12 per genotype.
DOI:
http://dx.doi.org/10.7554/eLife.10891.008