- Coenzyme Q10 as a possible treatment for neurodegenerative
diseases.
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- By MF Beal fbeal@mail.med.cornell.edu
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- Department of Neurology and Neuroscience, Weill Medical
College of Cornell University, New York Presbyterian Hospital, NY 10021
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- Coenzyme Q10 (CoQ10) is an essential cofactor of the
electron transport gene as well as an important antioxidant, which is particularly
effective within mitochondria.
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- A number of prior studies have shown that it can exert
efficacy in treating patients with known mitochondrial disorders.
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- We investigated the potential usefulness of coenzyme
Q10 in animal models of Parkinson's disease (PD), amyotrophic lateral sclerosis
(ALS) and Huntington's disease (HD).
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- It has been demonstrated that CoQ10 can protect against
striatal lesions produced by the mitochondrial toxins malonate and 3-nitropropionic
acid. These toxins have been utilized to model the striatal pathology,
which occurs in HD.
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- It also protects against 1-methyl-1,2,3,6-tetrahydropyridine
(MPTP) toxicity in mice.
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- CoQ10 significantly extended survival in a transgenic
mouse model of ALS.
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- CoQ10 can significantly extend survival, delay motor
deficits and delay weight loss and attenuate the development of striatal
atrophy in a transgenic mouse model of HD. In this mouse model, it showed
additive efficacy when combined with the N-methyl-D-aspartate (NMDA) receptor
antagonist, remacemide.
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- CoQ10 is presently being studied as a potential treatment
for early PD as well as in combination with remacemide as a potential treatment
for HD. Publication Types: Review Review, Tutorial PMID: 12069110
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- *******************************
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- Flint Beal, M.D., Ph.D., an experimental neurologist
at Harvard University Medical School, has echoed Dr. Perlmutter,s vision
of the future of neurology in his paper, Aging, Energy, and Oxidative Stress
in Neurodegenerative Disease. (1) Beal MF. Aging, energy, and oxidative
stress in neurodegenerative diseases. Ann Neurol. 1995 Sep; 38(3): 357-66.
Review. PMID: 7668820 Albers DS, Beal MF. Mitochondrial dysfunction and
oxidative stress in aging and neurodegenerative disease. J Neural Transm
Suppl. 2000; 59: 133-54. Review. PMID: 10961426 fbeal@mail.med.cornell.edu
http://www.med.cornell.edu/gradschool/fac/beal.html "Professor of
Neuroscience. Program Chair, Neuroscience. B.A. 1975, Colgate University,
M.D., 1976, University of Virginia. NEUROCHEMISTRY LABORATORY
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- The research in our laboratory investigates fundamental
mechanisms of cell death in neurodegenerative diseases. We have principally
focused on the role of mitochondrial dysfunction and oxidative damage in
neurodegenerative diseases. Our studies have utilized cell culture models,
transgenic animal models, mitochondrial toxin animal models, postmortem
neurochemical studies, studies of oxidative damage markers in body fluids
of patients, and MRI spectroscopy. Our laboratory has developed a large
number of sensitive and specific biochemical assays for markers of oxidative
damage. We have also developed techniques to measure a large number of
energy related metabolites. These techniques have been utilized to investigate
transgenic and other animal models with relevance to Huntington's disease,
amyotrophic lateral sclerosis, Parkinson's disease and Alzheimer's disease.
We have also investigated a number of novel therapeutic interventions related
to attempts to buffer intracellular energy stores and to prevent oxidative
damage."
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