BiP and CHOP upregulated in post-mortem brains from HD patients and in HD cell models (Duennwald and Lindquist, 2008; Carnemolla et al., 2009). BIM upregulated in HD animal and cells models (García-Martínez et al., 2007; Kong et al., 2009; Leon et al., 2010).XBP-1 upregulated in striatum of HD patients (Vidal et al., 2012).XBP-1 knock down reduced neuron loss and mHtt levels, and improved motor performance in HD mouse models (Vidal et al., 2012). Here, HSPA5 is linked to Huntington disease.