Article
Pathological mitophagy disrupts mitochondrial homeostasis in Leber's hereditary optic neuropathy.
Cell reports - 19 Jul 2022
Danese Alberto, Patergnani Simone, Maresca Alessandra, Peron Camille, Raimondi Andrea, Caporali Leonardo, Marchi Saverio, La Morgia Chiara, Del Dotto Valentina, Zanna Claudia, Iannielli Angelo, Segnali Alice, Di Meo Ivano, Cavaliere Andrea, Lebiedzinska-Arciszewska Magdalena, Wieckowski Mariusz R, Martinuzzi Andrea, Moraes-Filho Milton N, Salomao Solange R, Berezovsky Adriana, Belfort Rubens, Buser Christopher, Ross-Cisneros Fred N, Sadun Alfredo A, Tacchetti Carlo, Broccoli Vania, Giorgi Carlotta, Tiranti Valeria, Carelli Valerio, Pinton Paolo
Abstract excerpt
Leber's hereditary optic neuropathy (LHON), a disease associated with a mitochondrial DNA mutation, is characterized by blindness due to degeneration of retinal ganglion cells (RGCs) and their axons, which form the optic nerve. We show that a sustained pathological autophagy and compartment-specific mitophagy activity affects LHON patient-derived cells and cybrids, as well as induced pluripotent-stem-cell-derived...
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