PRNP and early-onset autosomal dominant Alzheimer disease: Most interestingly, a wealth of recent studies has established that PrP interacts with Amyloid β protein (Aβ), which is generated by the abnormal processing of the amyloid precursor protein (APP) by β-secretase, β-site APP cleaving enzyme (BACE1) and involved in the pathogenesis of Alzheimer's disease (Larson et al., 2012; Um et al., 2012; Um and Strittmatter, 2013; Dohler et al., 2014).