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 cellula

SUBMITTER: Jimenez-Loygorri JI 

PROVIDER: S-EPMC12686442 | biostudies-literature | 2025 Dec

REPOSITORIES: biostudies-literature