Article
Inhibition of autophagy curtails visual loss in a model of autosomal dominant optic atrophy.
Nature communications - 12 Aug 2020
Zaninello Marta, Palikaras Konstantinos, Naon Deborah, Iwata Keiko, Herkenne Stephanie, Quintana-Cabrera Ruben, Semenzato Martina, Grespi Francesca, Ross-Cisneros Fred N, Carelli Valerio, Sadun Alfredo A, Tavernarakis Nektarios, Scorrano Luca
Abstract excerpt
In autosomal dominant optic atrophy (ADOA), caused by mutations in the mitochondrial cristae biogenesis and fusion protein optic atrophy 1 (Opa1), retinal ganglion cell (RGC) dysfunction and visual loss occur by unknown mechanisms. Here, we show a role for autophagy in ADOA pathogenesis. In RGCs expressing mutated Opa1, active 5' AMP-activated protein kinase (AMPK) and its autophagy effector ULK1 accumulate at...
Read the complete abstract on PubMedTopics
Share this publication in a Topic to start or enrich a Post.