PRNP and amyloidosis: WH1(A31V)-mCh amyloidosis also shares significant similarities with the cytotoxicity pathways described for PrP in transmissible spongiform encephalopathies: (i) the generation of ROS in glial cells by NAD(P)H oxidase (NOX2) in the respiratory chain (Sorce et al., 2014); and (ii) the expression of genes involved in iron homeostasis (Hwang et al., 2009).