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Potential for creatine and other therapies targeting cellular energy dysfunction in neurological disorders

Tarnopolsky, Mark A., Beal, M. Flint·2001·Annals of Neurology

Abstract

Substantial evidence indicates that bioenergetic dysfunction plays either a primary or secondary role in the pathophysiology of cell death in neurodegenerative and neuromuscular disorders, and even in normal aging. Agents that ameliorate bioenergetic defects may therefore be useful in therapy. Creatine, which increases muscle and brain phosphocreatine concentrations, and may inhibit the activation of the mitochondrial permeability transition, protects against neuronal degeneration in transgenic murine models of amyotrophic lateral sclerosis and Huntington's disease and in chemically mediated neurotoxicity. Initial studies of creatine use in humans appear promising; however, further long‐term, well‐designed trials are needed. Coenzyme Q 10 , Gingko biloba , nicotinamide, riboflavin, carnitine, lipoic acid, and dichloroacetate are other agents which may have beneficial effects on energy metabolism, but the preclinical and clinical evidence for efficacy in neurological diseases remains limited. These compounds are widely used as dietary supplements; however, they must be subjected to rigorous evaluation through randomized, double‐blinded trials to establish efficacy, cost‐effectiveness and safety in neurological disorders.

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Cited by

Metadata

Authors
Tarnopolsky, M. A., & Beal, M. F.
Venue
Annals of Neurology
Publisher
Wiley
Published
2001-04-26
Volume
49
Issue
5
Pages
561-574
ISSN
  • 0364-5134
  • 1531-8249
Cited by
195
References
29
Language
en

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