Moreover, in a mouse model of myotonic dystrophy, BG and not neurons appeared to be the preferential target of RNA toxicity in the cerebellum, leading to EAAT2 downregulation, increased glutamate neurotoxicity, and PC firing abnormalities (Sicot et al., 2017).One of the most accredited pathogenic hypotheses in cerebellar ataxias, including SCA28, explains the degeneration of PCs with excitotoxicity, caused by excessive glutamatergic stimulation. The gene discussed is SLC1A2; the disease is cerebellar ataxia.