Do Mitochondria play a part?
Mitochondria are small bodies that act as the powerhouses of every cell. They supply energy crucial to cell survival. Recent theories suggest that mutant SOD1 may interfere with the functioning of mitochondria within nerve cells. If that’s the case, steps that boost the activity of mitochondria might improve ALS patients’ condition. It’s this idea, in part, behind the experimental use of creatine—a basic "fuel" mitochondria use to make energy— in ALS models. The modest improvements in survival of mouse models on high creatine diets has led to its widespread use by ALS patients worldwide.

Currently, researchers at the Center and other laboratories in this country are running clinical trials to evaluate creatine’s usefulness more scientifically. In addition, several Center investigators are trying to see if dysfunction in mitochondria has any tie to mutant SOD1 proteins.

These studies have special importance because a major theory of what goes awry in ALS—the overactivity of a nerve-stimulating molecule called glutamate—is also a step on the same path to nerve cell death that includes mitochondria. Glutamate toxicity is known to be common in nerve cells of ALS patients and in ALS models.