OPA1 encodes for an inner mitochondrial membrane protein that regulates a host of important functions, including the balance between mitochondrial fusion and fission, the stability of the mitochondrial respiratory chain complexes, and the controlled release of proapoptotic cytochrome c molecules sequestered within the mitochondrial cristae.4 DOA is therefore a mitochondrially determined optic neuropathy characterized by the preferential loss of retinal ganglion cells within the inner retina, similar to Leber hereditary optic neuropathy (LHON). Here, OPA1 is linked to optic nerve disorder.