The scientists focused on the protein ataxin-3. When it mutates, ataxin-3 begins to aggregate and form fibrils with devastating consequences on motor control and coordination. This neurodegenerative disease is known as spinocerebellar ataxia.
Scientists showed that ataxin-3 misfold after it aggregates, not before as would be expected by current views on aggregation. In fact, the aggregation of ataxin-3 seems to begin with the individual protein, and then moves onto the formation of intermediate aggregation forms with the original protein structure rather than a misfolded one.