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Model of the ataxin-3 aggregation mechanism. A, location of the identified amyloidogenic peptides (from Fig. 2), regions that displayed a polyQ-dependent increase in dynamics (from Fig. 3), and alanine-scanning mutations (from Fig. 5) within the Josephin domain (PDB code 1YZB (22)). B, schematic representation of the role of polyQ tract expansion in triggering ataxin-3 misfolding and aggregation.
