Evaluation of Mitochondrial Dysfunction and Idebenone Responsiveness in Fibroblasts from Leber’s Hereditary Optic Neuropathy (LHON) Subjects

• Published in: International journal of molecular sciences Vol. 24; no. 16; p. 12580
• Main Authors: Baglivo, Mirko, Nasca, Alessia, Lamantea, Eleonora, Vinci, Stefano, Spagnolo, Manuela, Marchet, Silvia, Prokisch, Holger, Catania, Alessia, Lamperti, Costanza, Ghezzi, Daniele
• Format: Journal Article
• Language: English
• Published: Basel MDPI AG 08.08.2023; MDPI
• Subjects: Analysis; Biosynthesis; Cell growth; Communication; Disease; Fibroblasts; Mitochondrial DNA; Respiration

Leber’s hereditary optic neuropathy (LHON) is a disease that affects the optical nerve, causing visual loss. The diagnosis of LHON is mostly defined by the identification of three pathogenic variants in the mitochondrial DNA. Idebenone is widely used to treat LHON patients, but only some of them are...