TARDBP and Machado-Joseph disease: To this end, we utilized the abovementioned aggregation-prone TDP-43-YFP (Fig 6E, upper) as well as human Ataxin3 containing a pathogenic expansion of 78 glutamine repeats (Machado-Joseph disease truncated-polyglutamine 78 [MJDtr-Q78]; Fig 6E, bottom), which also causes retinal degeneration when overexpressed in Drosophila eyes [32] (Fig 6E, bottom).