C2-8

C2-8 is an inhibitor of polyglutamine (polyQ) aggregation with IC50s of 25 and 0.05 μM for recombinant HDQ51 and in PC12 cells, respectively. It also limits polyQ aggregation in organotypic hippocampal slice cultures from R6/2 transgenic mice and reduces neurodegeneration dose-dependently in a Drosophila model of Huntington's disease. C2-8 (100 and 200 mg/kg) decreases huntingtin aggregate size, reduces neuronal atrophy, and enhances motor performance in a rotarod test in the R6/2 transgenic mouse model of Huntington's disease.