Figure 1

Neither basal nor activated levels of HSF1 change with disease progression in zQ175 tibialis anterior, striatum or cortex. On the left-hand side, western blot images showing HSF1 protein (top) or ATP5B (bottom) levels in tibialis anterior, striatum and cortex of zQ175 and wild-type mice treated with NVP-HSP990 or vehicle (n = 5–6/genotype/treatment) at 20 months of age. HSF1 antibody 51034-1-AP (Proteintech) was used in cortex and tibialis anterior and antibody A303-176A (Bethyl Laboratories) in striatum. Quantification was performed separately for the vehicle and NVP-HSP990 treated samples and normalised to wild-type in each case (right-hand side). There were no statistically significant differences between the genotypes for either treatment. Statistical analysis was by unpaired Student’s t-test. Mean ± SEM. The test statistics can be found in Supplementary Table S6 online. Uncropped blots are presented in Supplementary Figs. S2–S4 online. Veh vehicle, 990 NVP-HSP990, WT wild-type, m months, KO knockout, ant. anterior.