Several previous observations have demonstrated that the expression level of multiple heat shock protein (HSP) genes are upregulated in polyQ diseases (Huen and Chan, 2005; Huen et al., 2007; Tagawa et al., 2007), and such gene induction events are considered to be a cellular protective mechanism aiming to neutralize protein toxicity through promoting refolding of the polyQ disease protein. The gene discussed is HSP90B2P; the disease is glycogen storage disease VI.