Journal of Neurochemistry Publishes Article - Co-enzyme Q10 and Idebenone use in Friedreich's Ataxia
Friedreich's ataxia is a debilitating progressive neurodegenerative disease associated with cardiomyopathy and other features. The underlying cause is a deficiency of the mitochondrial protein frataxin which causes mitochondrial iron deposition, increased oxidative stress and impaired adenosine triphosphate production. Over the last 15 years, multiple clinical trials have assessed the efficacy of antioxidant agents in this disease. This article reviews trials of the two most important agents, namely co-enzyme Q10 and idebenone.....see full article
What is Friedreich's Ataxia?
Friedreich's ataxia is a neuro-degenerative disease in which the mitochondria in the cells are progressively destroyed as free radicals attack them. In its early stages Friedreich's is characterized by loss of balance while standing and walking and slowly progresses to wheelchair dependency. It often results in premature death. It presents itself by the late teens or early twenties. Slurred speech is another early symptom of Friedreich's as are heart abnormalities. It is genetic and progressive. Sometimes doesn't present itself until 25 and takes another 15 years to become full blown. Early or late, the symptoms are the same. Their spinal cord has begins to thin, their vision has begun to blur, hearing is impaired, speech slurs, scoliosis (curvature of the spine) has presented itself. Heart disorders ensue. About 20% of Friedreich's sufferers develop a carbohydrate intolerance and diabetes.
Idebenone treatment in early Friedreich's patients.
All cells contain many tiny organs called mitochondria. Mitochondria are the factories that give us our energy. Young or old, healthy mitochondria make us feel young and vital. It can be said that well-functioning mitochondria are the true fountain of youth. All mitochondria contain a protein called frataxin. Friedreich's manifests when unpaired free radicals progressively attack mitochondria causing frataxin to erode. This causes dysfunction in the nerve tissue of the heart, liver, kidney, brown fat, an endless list, to degenerate. This degeneration attacks the spinal cord, nerve tissues heart even in healthy people. For those with the Friedreich's gene, in is far worse, impairing muscle coordination. The arms and legs are the first to be hit in children as young as 15. Artuch R, Aracil and colleagues at the Biochemistry Department, in the University of Barcelona, Spain analyzed frataxin genes and found a significant improvement in frataxin loss after introducing idebenone after only 3 months of treatment. Cerebellar improvement was vastly improved as was the progression of the disease. Dr. Rotig A, Sidi Munnich A, of the Cardiologie pediatrique Hospital in Paris confirmed this by finding that oxidative insult to frataxin resulted in disabled recruitment of early antioxidant defenses and that Idebenone therapy counteracted the course of the disease. Meier T, Buyse G. Santhera Pharmaceuticals in Switzerland. summarized the results of idebenone treatment studies published over a ten year period (1999 to 2008) and found that it positively influenced the electron balance in mitochondria, In vitro studies showed that idebenone is a superior electron carrier that fostered (ATP) adenosine triphosphate production as well. Idebenone is extremely well tolerated at high doses. The fastest delivery method for idebenone is via liposomal cream. Though it has not been studied in relation to Ataxia another excellent way to rejuvenate mitochondria is with Alpha Lipoic Acid. It is an excellent liver detoxifiers that also pulls sugar out of the plasma and puts it in the mitochondria where it is converted and burnt off as energy.