The causes of motor neuron death in amyotrophic lateral sclerosis (ALS) are so far
unknown. The involvement of mitochondria in the disease was initially suggested by
ultrastructural studies. More recently these observations have been supported by studies of
mitochondrial function in ALS. Alterations in the activity of complexes which make up the
mitochondrial electron transport chain have been recorded as well as mutations in the
mitochondrial genome. The calcium buffering function of the mitochondria may also be …