OPA1 product is a nuclear-encoded mitochondrial protein with similarity to dynamin-related GTPases. It is a component of the mitochondrial network. Highly expressed in the retina, OPA1 mutations have been associated with a dominantly inherited optic neuropathy, called optic atrophy type 1, resulting in progressive loss of visual acuity. Eight transcript variants encoding different isoforms, resulting from alternative splicing of exon 4 and two novel exons named 4b and 5b, have been reported for this gene.

OPA1 antibodies are used primarily for vision research.