FIGURE 1.

HDAC6 co-localizes with DPR pathology in c9FTD/ALS. (A) Representative images of immunohistochemical analysis of HDAC6 in frontal cortex (top panels) and hippocampus (bottom panels) from control, sporadic FTD/ALS, or c9FTD/ALS patients (n = 5 per group). Arrowheads indicate HDAC6 inclusions. Scale bars, 20 μm. (B) Triple-immunofluorescence staining for HDAC6, poly (GR), and either poly (GA) (top panel) or poly (GP) (bottom panel) in the hippocampus of c9FTD/ALS patients. Scale bars, 10 μm. (C) Quantitative analysis of the percentage of HDAC6-positive inclusions that co-localize with poly (GA), poly (GP) and poly (GR) in the hippocampus of c9FTD/ALS patients (n = 5). Data are presented as mean ± SEM, ****p < 0.0001, one-way ANOVA, Tukey’s multiple-comparison test. (D) HEK293T cells were transfected with myc-HDAC6 and GFP, GFP-(GA)₅₀, or GFP-(GP)₄₇. GFP and myc-HDAC6 levels were evaluated in cell lysates by immunoblotting (left panel), with GAPDH used to control for protein loading. Myc-HDAC6 or GFP were immunoprecipitated from cell lysates, followed by immunoblotting for myc or GFP (right panel).