Despite the efforts of different research teams towards testing different molecular strategies to treat SCA3/MJD—such as chaperone induction [7], activation of autophagy [7,8,9,10], caloric restriction [11], modulation of intracellular calcium homeostasis [12], gene silencing [13,14,15,16], antioxidant treatment [17], HDAC inhibition [18,19], increasing serotonergic signaling [20], and neural stem cell transplantation [21]—an effective treatment to halt the progression of this fatal disease is not yet available (reviewed in [22]). Here, HDAC9 is linked to Spinocerebellar ataxia type 3.