Figure 4.
Inhibition of HD exon 1 aggregation in 293 Tet-Off cells. (A) Structure of chemical compounds counteracting HDQ51 aggregation in vivo. (B) Quantification of filter retardation assay results. Cells were incubated for 72 h in the presence of various concentrations of the indicated chemical compounds. Protein extracts were prepared and filtered through a cellulose acetate membrane; captured SDS-insoluble protein aggregates were detected with the HD1 antibody. The dots corresponding to the control reactions without added chemical compound (not shown) were arbitrarily set as 100. (C) Relative protein concentrations of the cell extracts analyzed in B. (D) Western blot of cell extracts prepared from 293 Tet-Off cells after treatment with increasing concentrations of the chemical compound PGL-135 and the solvent DMSO. The arrow indicates the HDQ51 monomer.