Journal
FREE RADICAL BIOLOGY AND MEDICINE
Volume 63, Issue -, Pages 1-29Publisher
ELSEVIER SCIENCE INC
DOI: 10.1016/j.freeradbiomed.2013.03.018
Keywords
Parkinson's disease; Alzheimer's disease; Huntington's disease; Amyotrophic lateral sclerosis; Charcot-Marie-Tooth disease and Friedreich's ataxia; Neurodegenerative diseases; Mitochondrial dysfunction; Creatine; Co-Q10; PGC-1 alpha; Sirtuins; Free radicals
Funding
- NINDS, NIA
- Department of Defense
- BSC0115 MiND grant
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Neurodegenerative disorders are debilitating diseases of the brain, characterized by behavioral, motor and cognitive impairments. Ample evidence underpins mitochondrial dysfunction as a central causal factor in the pathogenesis of neurodegenerative disorders including Parkinson's disease, Huntington's disease, Alzheimer's disease, Amyotrophic lateral sclerosis, Friedreich's ataxia and Charcot-Marie-Tooth disease. In this review, we discuss the role of mitochondrial dysfunction such as bioenergetics defects, mitochondrial DNA mutations, gene mutations, altered mitochondrial dynamics (mitochondrial fusion/fission, morphology, size, transport/trafficking, and movement), impaired transcription and the association of mutated proteins with mitochondria in these diseases. We highlight the therapeutic role of mitochondrial bioenergetic agents in toxin and in cellular and genetic animal models of neurodegenerative disorders. We also discuss clinical trials of bioenergetics agents in neurodegenerative disorders. Lastly, we shed light on PGC-1 alpha, TORC-1, AMP kinase, Nrf2-ARE, and Sirtuins as novel therapeutic targets for neurodegenerative disorders. (C) 2013 Elsevier Inc. All rights reserved.
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