Huntington’s disease (HD) results from the accumulation of the intracellular protein huntingtin in the striatum of the neurons causing an increase in the expression of BiP and CHOP in the parietal cortex [81] and an increase in BiP expression and phosphorylation of IRE1 in striatal tissues from HD mice brains [82]. The gene discussed is HSPA5; the disease is juvenile Huntington disease.