The Role of Mitochondria in Amyotrophic Lateral Sclerosis.

•Mitochondrial dysfunction is one of the earliest pathophysiological events in amyotrophic lateral sclerosis (ALS).•ALS-associated mutant proteins accumulate in mitochondria and cause mitochondrial damage.•ALS-associated mitochondrial dysfunction occurs at multiple levels.•ALS affects mitochondrial respiration and ATP production, calcium handling, dynamics, and apoptotic signalling.