We show here that over-expression of Oxr1 in the cytoplasm is capable of preventing the mitochondrial morphological changes induced by rotenone, while a reduction of mitochondrial length is observed in primary GCs lacking Oxr1. These data are in line with our recent observations that Oxr1 can prevent mitochondrial morphology defects caused by ALS-associated Tdp-43 mutations [13]. Here, OXR1 is linked to amyotrophic lateral sclerosis.