In neurological diseases due to altered mitochondrial dynamics, such as type 2A Charcot-Marie-Tooth with pathogenic MFN2 variants and dominant optic atrophy (ADOA) with pathogenic OPA1 variants, some defects in OXPHOS coupling and/or altered MRC activity have been found in mouse models14 and primary fibroblasts from patients,15,16 while the reverse connection linking OXPHOS activity and the network morphology, remains more obscure. This evidence concerns the gene OPA1 and autosomal dominant optic atrophy.