In human brain tissue activation of the UPR has been detected in several neurodegenerative diseases including Alzheimer’s disease (AD), frontotemporal lobar degeneration with tau pathology (FTLD-tau), Parkinson’s disease (PD) and amyotrophic lateral sclerosis (ALS) (for review see [23]). This evidence concerns the gene MAPT and early-onset autosomal dominant Alzheimer disease.