Interestingly, this analysis identified numerous genes in which mutations are known to cause Mendelian forms of ataxia namely, ANO10 (Vermeer et al., 2010; Mišković et al., 2016), CABC1 (Mollet et al., 2008; Gerards et al., 2010) and POLG (Schicks et al., 2010; Synofzik et al., 2012) that were significantly down-regulated in the heart tissue and slightly down-regulated in the cerebellum after Fxn knockdown in Tg + animals (Figure 8c and Figure 8—figure supplement 1). Here, COQ8A is linked to cerebellar ataxia.