The levels of antioxidant enzymes such as catalase, superoxide dismutase 2 (SOD2), and other oxidative stress markers were shown to be dysregulated in cellular models for SCA2, SCA3/MJD and SCA7 [33, 151, 165–168], in an SCA7 mouse model [169] and in SCA2, SCA3/MJD and SCA7 patient samples [170–172]. Here, SOD2 is linked to Machado-Joseph disease.