Idebenone is a synthetic analogue of ubiquinone (also known as Coenzyme Q10), a vital cell antioxidant and essential component of the Electron Transport Chain (ETC). It has been proposed that by interacting with the ETC, idebenone increases ATP production required for mitochondrial function, reduces free radicals, inhibits lipid peroxidation, and consequently protects the lipid membrane and mitochondria from oxidative damage . More specifically, idebenone is thought to transfer electrons directly to complex III of the mitochondrial ETC, thereby circumventing complex I and restoring cellular energy (ATP) generation .
Due to its ability to reduce oxidative damage and improve ATP production, idebenone was originally investigated for its potential use in Alzheimer's Disease and other cognitivie disorders . Lack of improvement in cognitive function halted its production for these conditions, however it continues to be investigated for use in other conditions associated with mitochondrial damage.
Idebenone is currently only indicated for use by the European Medicines Agency (EMA) for the treatment of visual impairment in adolescent and adult patients with
Leber’s Hereditary Optic Neuropathy (LHON). LHON is a mitochondrially inherited degeneration of retinal ganglion cells, resulting in acute central vision loss. Due to its biochemical mode of action, it's thought that idebenone may re-activate viable-but-inactive retinal ganglion cells (RGCs) in LHON patients . It is not currently approved for use by either the Food and Drug Administration (USA) or Health Canada.
Idebenone is indicated for use by the European Medicines Agency (EMA) for the treatment of visual impairment in adolescent and adult patients with
Leber’s Hereditary Optic Neuropathy (LHON). It is not currently approved for use by either the Food and Drug Administration (USA) or Health Canada .
Eurofins Optimed, Gières, France
Johns Hopkins University, Baltimore, Maryland, United States
Rare Disease Research, Atlanta, Georgia, United States
Childrens Hospital of Los Angeles, Los Angeles, California, United States
University Hospital of Wales, Cardiff, United Kingdom
Institut Catala de Retina, Barcelona, Spain
Hospital Universitario Ramon y Cajal, Madrid, Spain
Ospedale San Raffaele, Milano, Italy
Universitaetsklinikum Essen, Essen, Germany
CHU Amiens - Centre Saint Victor, Amiens, Somme, France
National Institutes of Health Clinical Center, 9000 Rockville Pike, Bethesda, Maryland, United States
The National Hospital, University College London, London, United Kingdom
University of California Davis Medical Center, Sacramento, California, United States
Children's Hospital Colorado, Aurora, Colorado, United States
Carolinas Medical Center, Neurosciences and Spine Institute, Charlotte, North Carolina, United States
Hôpital de la Salpétrière - INSERM U679, Neurologie et Thérapeutique expérimentale, Paris, France
Zentrum für Neurologische Medizin, Göttingen, Germany
HELIOS Klinikum Berlin, Berlin, Germany
National Institutes of Health Clinical Center, 9000 Rockville Pike, Bethesda, Maryland, United States
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