APP and Alzheimer disease: Due to the accumulation and aggregation of Aβ in mitochondria, as well as impaired mitochondrial function, it is not surprising that the UPRmt was also found to be upregulated in FAD and SAD [160,161], in cells overexpressing APP and in AD mouse models [161], as manifested by the increased levels of Hsp-60, Grp75, ClpP and Lon.