Interestingly, mutations in fusion components are linked to neurodegenerative disorders with Opa1 mutations causative for dominant optic atrophy [18] and mutations in Mfn2 linked to Charcot-Marie-Tooth type 2A disease, a peripheral neuropathy sometimes accompanied by optic degeneration and hearing loss [19]. Here, OPA1 is linked to Charcot-Marie-Tooth disease type 2A1.