Despite the growing interest in USP14 inhibition as a treatment for neurological diseases ([11, 17] but see [18]) and cancers [13, 14, 19, 20], loss of USP14 in the spontaneously occurring ataxia (axJ) mouse mutant causes a severe loss of mobility and early postnatal lethality [21]. The gene discussed is USP14; the disease is Ataxia.