Fig. 1

Differential Htt pathology between HD striatum and cortex. Representative immunoblots of endogenous full-length Huntingtin (Htt) (1HU-4C8 antibody) and N-terminal Htt fragments (1HU-4C8 antibody) in the soluble fraction of A the striatum or B the cortex of HD patients and Controls. C–E Protein quantifications of soluble full-length Htt (1HU-4C8 and CH00146 antibodies) and N-terminal Htt (1HU-4C8 antibody). Representative dot blots of formic acid (FA)-insoluble full-length Htt (1HU-4C8) and mutant Htt (Poly-Gln) in F the striatum and G the cortex of HD patients and Controls. H, I Protein quantifications of dot blots. Representative immunoblots of endogenous NeuN, Darpp32 and PSD95 in J the striatum or K the cortex on HD patients and Controls with quantifications in (L, M). Bar graphs with standard error of the mean (SEM) are shown, where the average of Controls is set as 100%. In all cases, the HD group is presented as pooled or per stage. Statistics: Ctl vs. HD as a group was calculated using a Mann–Whitney test. Ctl vs. HD stages was calculated using an analysis of covariance followed by the Kruskal–Wallis multiple comparison test. Significant fold changes are provided for each group. * P < 0.05; **P < 0.01; ***P < 0.001; ****P < 0.0001. Abbreviations: Ctl, Controls; HD, Huntington’s disease; HD2, Vonsattel grade 2; HD3, Vonsattel grade 3; HD4, Vonsattel grade 4