The protective effect of erinacine A–enriched Hericium erinaceus mycelium ethanol extract on oxidative Stress–Induced neurotoxicity in cell and Drosophila models of spinocerebellar ataxia type 3

• HEME diminishes BH induced apoptosis in SK-N-SH-MJD78 cells. • HEME decreases mutant ataxin-3 protein aggregation in tBH treated SCA3 cell model. • HEME modulates p53 and NF-κB activation in tBH treated SK-N-SH-MJD78 cells. • HEME increases survival and climbing ability in tBH treated SCA3 Flies.