Cargando…
Pathological mitophagy disrupts mitochondrial homeostasis in Leber’s hereditary optic neuropathy
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...
Autores principales: | 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 |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Cell Press
2022
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9314546/ https://www.ncbi.nlm.nih.gov/pubmed/35858578 http://dx.doi.org/10.1016/j.celrep.2022.111124 |
Ejemplares similares
-
Exploiting hiPSCs in Leber's Hereditary Optic Neuropathy (LHON): Present Achievements and Future Perspectives
por: Peron, Camille, et al.
Publicado: (2021) -
The relevance of mitochondrial DNA variants fluctuation during reprogramming and neuronal differentiation of human iPSCs
por: Palombo, Flavia, et al.
Publicado: (2021) -
Pharmacological Inhibition of Necroptosis Protects from Dopaminergic Neuronal Cell Death in Parkinson’s Disease Models
por: Iannielli, Angelo, et al.
Publicado: (2018) -
Peculiar combinations of individually non-pathogenic missense mitochondrial DNA variants cause low penetrance Leber’s hereditary optic neuropathy
por: Caporali, Leonardo, et al.
Publicado: (2018) -
Cigarette toxicity triggers Leber's hereditary optic neuropathy by affecting mtDNA copy number, oxidative phosphorylation and ROS detoxification pathways
por: Giordano, L, et al.
Publicado: (2015)