BACE1 and early-onset autosomal dominant Alzheimer disease: Being the rate-limiting enzyme in the amyloidogenic pathway and playing a large role in the production of the neurotoxic intermediate, Aβ, aberrant BACE1 activity is considered pivotal to Alzheimer’s disease progression (Hardy, 1992; Hardy and Higgins, 1992; Selkoe, 2004; Gandy, 2005; Hampel and Shen, 2009).