The NRF-2/PGC-1α double knockout (dKO) mouse model develops an AMD-like phenotype, exhibiting RPE degeneration, mitochondrial dysfunction, and increased level of markers of both OS (i.e., 4-Hydroxynonenal) and proteostasis failure (i.e., SQSTM1/p62, Beclin-1, LC3B) [68,69]. Here, SQSTM1 is linked to age-related macular degeneration.