In conclusion, these post-translational changes in VDAC1 may be involved in its specific interaction with ALS-related SOD1 mutants and it is reasonable to hypothesize a role of the modified isoform in the disease and as a biomarker of irreparably damaged mitochondria, and at the same time for the early diagnosis of this pathology. Here, VDAC1 is linked to amyotrophic lateral sclerosis.