Scientists speculate that the abnormal form of the gene may initiate a deluge of nerve cell death. The gene's exact method of execution is unknown but many scientists believe it may involve free radical production. Some scientists believe other long-standing theories about the cause of ALS also come into play. Researchers surmise that immune system abnormalities may help contribute to nerve cell death. In addition, an accumulation of proteins that normally serve as skeleton in the neuron may be implicated.
Many scientists also point to a substance known as glutamate as having a hand in neuron death.
This chemical excites cells and increases production of a destructive free radical. Researchers suspect the free radicals may continue the deadly cycle by causing more glutamate to be released. This research led to the first Food and Drug Administration approved drug for ALS, riluzole. The compound reduces glutamate-induced nerve cell damage. Recent studies show it can prolong the life of patients approximately three months.