Interestingly, mutation of AFG3L2, which encodes the m-AAA subunit of the mitochondrial metalloprotease AFG3L2 (ATPase family gene 3-like 2) has been found to underlie SCA28 (Di Bella et al., 2010; Almajan et al., 2012; König et al., 2016). The gene discussed is AFG3L2; the disease is spinocerebellar ataxia type 28.