We previously demonstrated increased mitophagy in fibroblasts from patients with Leber hereditary optic neuropathy (LHON).6 This was attenuated by idebenone, which conferred symptomatic improvement.6 To clarify whether increased mitophagy is an important feature of mitochondrial optic neuropathies, we investigated the role of OPA1 in mitophagy in primary OPA1 mutant fibroblasts from 5 patients in 3 families with severe DOA plus phenotypes. The gene discussed is OPA1; the disease is Leber hereditary optic neuropathy.