OXR1 has orthologues present in all eukaryotic organisms from yeast to humans and it has been demonstrated that the antioxidant properties of this protein are conserved throughout evolution [7], [8]; Oxr1 deletion in Saccharomyces cerevisiae increases sensitivity to hydrogen peroxide (H2O2) damage [7] and the mouse Oxr1 deletion mutant (bella) displays progressive ataxia, a shortened lifespan and cerebellar neurodegeneration [9]. Here, OXR1 is linked to cerebellar ataxia.