Overall, the findings extend our previous report that already the synthesis of the metabolite NAA from acetyl-CoA and aspartate by neuronal mitochondria, which is delivered to oligodendrocytes and crucial for myelin production, is impaired in the Atxn2-CAG100-KIN mouse as well as in SCA2 patients [51]. The gene discussed is ATXN2; the disease is spinocerebellar ataxia type 2.