Role of mitochondria in amyotrophic lateral sclerosis

Neurodegeneration in amyotrophic lateral sclerosis (ALS) is characterized b y the specific loss of central and peripheral motor neurons. While this pat tern of neuronal demise gives rise to a distinct clinical syndrome, at the cellular and molecular level ALS pathology is similar to that seen in other neurodegenerative diseases. In particular, mitochondrial dysfunction in AL S is reminiscent of that observed in Alzheimer's and Parkinson's diseases. Mitochondria in persons with ALS demonstrate impaired electron transport, i ncreased free radical generation, and an inability to adequately buffer cyt osolic calcium shifts. These abnormalities are probably systemic and potent ially due to mutation of mitochondrial DNA.