"Our study results are promising as they provide a foundation for evaluating drugs that could slow down the progression of ALS," says Dr. Friedlander, senior author of the study. "Although much more research needs to be conducted in this area, our research does bring us one step closer to finding a treatment for this tragic disease."
ALS is characterized by progressive loss of the motor neurons in the brain, brainstem, and spinal cord. On average, people who develop ALS die within five years of contracting the disease. Roughly 10 percent to 20 percent of ALS cases are hereditary.
All of the mice in the study were implanted with osmotic pumps that delivered zVAD-fmk, or a placebo, into the ventricles of the brain. They received the pumps at 60 days old, when the symptoms of the disease had not yet appeared, and received zVAD-fmk or placebo continuously for 56 days. The motor function of the mice was measured by timing how long they were able to stay on a rotating treadmill-like device called a Rotarod at a certain speed. The mice who received the highest doses of zVAD-fmk survived for an average of 153 days, as compared with 126 days for their untreated littermates, and were symptom-free for 20 days longer, on average.
The research was funded by the National Institute for Neurological Disorders and Stroke, the Muscular Dystrophy Association, the ALS Association, and Project ALS.