The role of mitochondria in amyotrophic lateral sclerosis

Abstract

Mitochondria are unique organelles that are essential for a variety of cellular processes including energy metabolism, calcium homeostasis, lipid biosynthesis, and apoptosis. Mitochondrial dysfunction is a prevalent feature of many neurodegenerative diseases including motor neuron disorders such as amyotrophic lateral sclerosis (ALS). Disruption of mitochondrial structure, dynamics, bioenergetics and calcium buffering has been extensively reported in ALS patients and model systems and has been suggested to be directly involved in disease pathogenesis. Here we review the alterations in mitochondrial parameters in ALS and examine the common pathways to dysfunction.

Keywords: Amyothrophic lateral sclerosis; Apoptosis; Axonal transport; Mitochondria; Mitophagy; Motor neuron disease; Neurodegeneration; Oxidative phosphorylation; Oxidative stress.