ATXN3 and Machado-Joseph disease: Previous evidence also suggested a protective role for VPA in the context of MJD, both in cell and Drosophila models, by attenuating mutant ATXN3 induced cell toxicity and alleviating polyQ-induced phenotypic abnormalities, without major impact on ATXN3 inclusion [19,20]; in C. elegans, there was some improvement of aggregation, but less prominent than that observed for other compounds, for instance Hsp90 inhibitors [21].