A new discovery from The Center of Free Radical and Biomedical Research at Facultad de Medicina, Universidad de la Republica in Uruguay may have direct application for patients with amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig’s disease. Patients with ALS have degenerated motor neurons, and they cannot control muscle movement, leading to paralysis and eventual death. Since motor neurons, just like all cells of the body, require an energy supply from mitochondria, researchers at The Center investigated a potential benefit of administering MitoQ to mice with ALS-like disease. MitoQ is a synthetic antioxidant that is analogous to Coenzyme Q10, a natural antioxidant in the body. It accumulates in the mitochondria and scavenges free radicals, minimizing oxidative stress damage in cells.
The team of scientists thought the antioxidant properties of MitoQ would attenuate damage to motor neurons and slow the progression of symptoms in ALS-induced mice. To test this theory, they administered MitoQ to mice via drinking water once the mice showed signs of neurodegeneration. After 20 days, all tested tissues of the mice had detectable levels of MitoQ, and the spinal cord and quadriceps muscles displayed signs of slower decline in mitochondrial function. Additionally, “MitoQ increased approximately 6% the survival of the treated mice,” noted Patricia Cassina, MD, PhD. “This might seem small, but it’s in the range of the best that other drugs have been able to achieve with this model in which the disease progression is extremely fast and there is a small window of opportunity to improve symptoms and survival.”
“We also found that MitoQ has beneficial effects in the murine model of ALS, which will likely lead to clinical trials using MitoQ with ALS patients and hopefully lead to extend the survival and improve the quality of life of ALS patients,” said Rafael Radi, MD, PhD, Director of The Center. Importantly, by waiting until symptoms presented, the scientists showed positive applicability to humans because patients would not seek treatment until their disease state became evident.
Also of note is the advancement of understanding in the world of ALS. “This study supports the role of mitochondrial dysfunction in the development and progression of ALS, which may allow for the development of more mitochondria-targeted therapies to fight this disease,” said Dr. Radi. A number of natural antioxidants are found in foods we eat, and perhaps they too can slow the progression of ALS. For now, it is certain that MitoQ will be studied further. It has been tested for safety in a number of unrelated clinical trials since its development in the late 1990s.