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| Goal tending | |
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Treatments for mitochondrial disorders, according to Peter W. Stacpoole, M.D., Ph.D., should be designed to address a minimum of two goals:  First, maximize the production of mitochondrial ATP
(adenosine triphosphate) by stimulating aerobic glucose oxidation. Second, minimize the production of free radicals by reversing cellular energy failure and increasing the efficiency of the body’s natural mechanisms for scavenging free radicals. Because mitochondria are especially vulnerable to attacks by free radicals, Stacpoole believes it is important in mitochondrial disorders to attempt to limit cell damage from free radicals. In his review article published in Metabolism, Stacpoole offers a biochemical rationale for several possible ways to address both of these goals. Treatment options discussed include:
carnitine dichloroacetate (DCA) vitamin C vitamin E beta-carotene omega-3 fatty acids.Additional investigations are required to evaluate these treatments. |
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| Reference: Stacpoole, PW; Lactic Acidosis and Other Mitochondrial Disorders Metabolism 1997 46:306-21 Related links: Carnitine and CoQ10: Miracles Cures or Money Down the Drain? MDA Quest article that explores these two cofactors. Levocarnitine Sigma-Tau site about Carnitor. Includes easy to understand explanations about what carnitine does, as well as a downloadable prescribing brochure in PDF (Adobe Acrobat Reader) format. New directions in mitochondrial disorders MDA update with comments from Salvatore DiMauro, M.D. Mitochondrial disease in perspective: Symptoms, diagnosis and hope for the future October 1999 Quest article that discusses free radicals, antioxidants, gene therapy, and other treatments for mitochondrial disorders. 
Mitochondrial
cytopathy in adults: What we know so far July 2001 review
by Bruce H. Cohen, M.D. and Deborah R. Gold, M.D. Note:
Requires Adobe Acrobat Reader to view. For more about
mitochondrial disorders, visit Sausages
and cells and Hot picks. For more about
free radicals and exercise, visit Radical
change.
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When some element of the mitochondrial assembly line is
defective, "raw" metabolic products begin to back up
(picture the famous candy factory episode of "I Love
Lucy"). Carnitine becomes bound to the extra metabolites
and delivers them to the kidney, where both compounds are
excreted in the urine. The net result of this activity is that
the overall amount of carnitine in the body can become reduced
because the kidneys excrete so much of the bound form.
