Defective chaperone-mediated autophagy in the retinal pigment epithelium of age-related macular degeneration patients

ABSTRACT: Autophagy is one of the main intracellular recycling systems and its impairment is considered a primary hallmark of the aging process. Defective macroautophagy in the retinal pigment epithelium (RPE) has been described in age-related macular degeneration (AMD), a blindness-causing disease that affects roughly 200 million patients worldwide. The relevance of chaperone-mediated autophagy (CMA), a selective type of autophagy for proteins containing a KFERQ-like motif, in RPE cell biology and homeostasis remains to be elucidated. Here we describe decreased CMA activity in the RPE of AMD patients compared to healthy age-matched controls, along with accumulation of substrate proteins, and in donor-derived iPSC-RPE cells which we used to further characterize AMD-associated alterations of cellular

SUBMITTER: Juan Ignacio Jiménez-Loygorri 

PROVIDER: S-SCDT-10_1038-S44321-025-00329-W | biostudies-other |

REPOSITORIES: biostudies-other