fig8

Figure 8. Proline mutation in fibril-forming motif inhibits the aggregation of expanded ataxin-3. (A) ThT binding assays of ataxin-3(45Q) and ataxin-3(45Q)/I80P. The kinetic curves are fitted with Hill function; (B) TEM measurements of ataxin-3(45Q) (left) and ataxin-3(45Q)/I80P (right); (C) Time-dependent SDS-PAGE analysis of sarkosyl-insoluble ataxin-3(45Q) and ataxin-3(45Q)/I80P. The scale bars represent 200 nm; (D) CD measurements of ataxin-3(45Q) (left) and ataxin-3(45Q)/I80P (right); (E) FTIR measurements of ataxin-3(45Q) (left) and ataxin-3(45Q)/I80P (right). TEM: Transmission electron microscopy; CD: circular dichroism; FTIR: Fourier transform infrared spectroscopy.