These findings are consistent with those demonstrated in murine models where Npc1−/− mice exhibit lower levels of HSP70 expression and HSF1 activation compared with wildtype mice; drug-induced elevated expression of HSP70 in the Npc1−/− mouse model was associated with reduction in glycosphingolipid accumulation, improvement in CNS myelination, improvement in measurable manifestations of ataxia and reduced loss of motor coordination [27]. Here, HSF1 is linked to cerebellar ataxia.