fig7

Figure 7. All residues in fibril-forming motif mutated to glycine inhibit the aggregation of non-expanded ataxin-3. (A) ThT binding assays of ataxin-3(22Q), ataxin-3(22Q)/I80A, and ataxin-3(22Q)/6G. The kinetic curves are fitted with Hill function; (B) TEM measurements of ataxin-3(22Q)/I80A (left) and ataxin-3(22Q)/6G (right); (C) Time-dependent SDS-PAGE analysis of sarkosyl-insoluble ataxin-3(22Q), ataxin-3(22Q)/I80A, and ataxin-3(22Q)/6G. The scale bars represent 200 nm; (D) CD measurements of ataxin-3(22Q)/I80A (left) and ataxin-3(22Q)/6G (right); (E) FTIR measurements of ataxin-3(22Q)/I80A (left) and ataxin-3(22Q)/6G (right). ThT: thioflavin T; TEM: transmission electron microscopy; CD: circular dichroism; FTIR: Fourier transform infrared spectroscopy.