In Charcot-Marie-Tooth disease (CMT), a peripheral neuropathy (Züchner et al. 2004), mutations in Mfn2 tilt the balance between mitochondrial fission and fusion, while mutated variants of Opa1 are thought to be the main cause of dominant optic atrophy (DOA), a degeneration of retinal ganglia cells, resulting in an atrophy of the optical nerve (Alavi et al. 2007). Here, MFN2 is linked to Charcot-Marie-Tooth disease.