Upregulation of UPR-responsive chaperones, such as Grp78/BiP, Grp94, and Grp58/ERp57, and other ER stress markers is observed in the brain of patients affected with sporadic and new variant CJD [30], [31] and in different mouse models of scrapie [22], [30], [32], [33], [34], [35]. This evidence concerns the gene HSPA5 and scrapie.