CoQ10 became linked to ALS due to associations between the nutrient, cellular mitochondria and motor neuron death. Amyotrophic lateral sclerosis, or ALS, is also known as motor neuron disease. Motor neurons are nerve cells that control muscles and they communicate by way of electrons being passed along what's known as the mitochondrial electron transport chain. In ALS, this chain is faulty and the neurons die, causing paralysis. Because CoQ10 helped patients with other neurodegenerative diseases, scientists hoped to find a similar benefit in ALS. It's imperative you seek your doctor's advice regarding possible supplementation for ALS.
Mitochondria
According to the ALS Association website, evidence is mounting that malfunctioning of cellular mitochondria explains why ALS selectively targets motor neurons. Mitochondria are the power plants of all animal cells and reside within cells providing fuel in the form of ATP or adenosine triphosphate. In the case of motor neurons, mitochondria sustain the capacity of a single cell to span from the spinal cord all the way to the toes by-way-of the electron transport chain. Studies have shown that in ALS, protein builds up in the mitochondria of motor neurons causing them to swell and burst apart.
CoQ10
CoQ10, sometimes known as coenzyme Q10, is an oil-soluble, vitamin-like substance primarily found in mitochondria. It's a critical component of the electron transport chain of mitochondria and a powerful free radical scavenger. According to researchers reporting in the January 2007 issue of "CNS Spectrums," CoQ10 is a promising neuroprotectant already proven to aid in repairing defects in the inner mitochondrial membranes of patients with other neurodegenerative disorders including Alzheimer's and Parkinson's diseases. CoQ10 also facilitates a process called oxidative phosphorylation, which sustains muscles through the production of ATP.
Safety First
Before testing CoQ10 in human ALS patients, a safety trial had to be conducted. Researchers reporting in the December 2005 issue of "Neurology" noted the importance of CoQ10 in mitochondrial function and discussed its success in protecting motor neurons in animal studies. Early-phase trial results of CoQ10 in other neurodegeneration diseases indicated that doses greater than 600 mg per day may be needed in humans. The researchers conducted a trial to assess the safety and tolerability of high doses of CoQ10 in ALS and found that 3,000 mg per day was safe and well tolerated for eight months.
Patient Trial
Researchers reporting in the August 2009 issue of the "Annals of Neurology" designed a two phase trial to test CoQ10's ability to help ALS patients. The first phase dosed ALS patients with 1,800 mg CoQ10 per day and the second used a 2,700 mg dose. The trial lasted nine months, and involved 110 patients randomized to receive either CoQ10 or placebo. The primary endpoint the researchers sought was an improvement in muscle function. In this respect, the CoQ10 failed; however, disease progression slowed. CoQ10 subjects declined less in physical and mental quality-of-life scores than did placebo subjects.
References
- ALS Association Research: Mitochondria
- "CNS Spectrums"; Coenzyme Q10: a Review of Its Promise as a Neuroprotectant; Joyce Young et al.; January 2007
- "Neurology"; Tolerance of High-Dose (3,000 Mg/Day) Coenzyme Q10 in ALS; Kyle Ferrante et al.; December 2005
- "Annals of Neurology"; Phase II Trial of CoQ10 for ALS Finds Insufficient Evidence to Justify Phase III; Petra Kaufmann et al.; August 2009
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