Therapeutic Options in Hereditary Optic Neuropathies

Medical Management of Hereditary Optic Neuropathies

Hereditary optic neuropathies are diseases affecting the optic nerve. The most common are mitochondrial hereditary optic neuropathies, i.e., the maternally inherited Leber's hereditary optic neuropathy (LHON) and dominant optic atrophy (DOA). They both share a mitochondrial pathogenesis that leads to the selective loss of retinal ganglion cells and axons, in particular of the papillo-macular bu...

Oxydative Metabolism in Optic Nerve Myelin: New Perspectives in Hereditary Optic Neuropathies

ANT Adenosine Nucleotide Translocase; BSA Bovine Serum Albumin; CLSM Confocal Laser Scanning Microscopy; CNS Central Nervous System; EGTA Ethylene Glycol Tetraacetic Acid; ETC Electron Transport Chain; GA Glutaraldehyde; Ide, Idebenone; IM Isolated Myelin; MBP Myelin Basic Protein; LHON LeberHereditary Optic Neuropathy; MW MolecularWeight; NS Nervous System; OXPHOS Oxidative Phosphorylation; PB...

Mitochondrial optic neuropathies – Disease mechanisms and therapeutic strategies

Leber hereditary optic neuropathy (LHON) and autosomal-dominant optic atrophy (DOA) are the two most common inherited optic neuropathies in the general population. Both disorders share striking pathological similarities, marked by the selective loss of retinal ganglion cells (RGCs) and the early involvement of the papillomacular bundle. Three mitochondrial DNA (mtDNA) point mutations; m.3460G>A...

Medical Rehabilitation in Hereditary Neuropathies

Mitochondrial optic neuropathies.