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Mitochondrial diseases of the brain.
Free Radic Biol Med. 2013 Oct; 63:1-29.FR

Abstract

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-1α, TORC-1, AMP kinase, Nrf2-ARE, and Sirtuins as novel therapeutic targets for neurodegenerative disorders.

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Publisher Full Text

Authors+Show Affiliations

Chaturvedi RK
CSIR-Indian Institute of Toxicology Research, 80 MG Marg, Lucknow 226001, India. rajnish@iitr.res.in
Flint Beal M
No affiliation info available

MeSH

Alzheimer DiseaseAmyotrophic Lateral SclerosisBrainCharcot-Marie-Tooth DiseaseFree RadicalsFriedreich AtaxiaHumansHuntington DiseaseMitochondriaMitochondrial DiseasesNerve DegenerationParkinson Disease

Pub Type(s)

Journal Article
Research Support, N.I.H., Extramural
Research Support, Non-U.S. Gov't
Research Support, U.S. Gov't, Non-P.H.S.
Review

Language

eng

PubMed ID

23567191

Citation

Chaturvedi, Rajnish K., and M Flint Beal. "Mitochondrial Diseases of the Brain." Free Radical Biology & Medicine, vol. 63, 2013, pp. 1-29.
Chaturvedi RK, Flint Beal M. Mitochondrial diseases of the brain. Free Radic Biol Med. 2013;63:1-29.
Chaturvedi, R. K., & Flint Beal, M. (2013). Mitochondrial diseases of the brain. Free Radical Biology & Medicine, 63, 1-29. https://doi.org/10.1016/j.freeradbiomed.2013.03.018
Chaturvedi RK, Flint Beal M. Mitochondrial Diseases of the Brain. Free Radic Biol Med. 2013;63:1-29. PubMed PMID: 23567191.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Mitochondrial diseases of the brain. AU - Chaturvedi,Rajnish K, AU - Flint Beal,M, Y1 - 2013/04/06/ PY - 2011/08/22/received PY - 2013/03/21/revised PY - 2013/03/22/accepted PY - 2013/4/10/entrez PY - 2013/4/10/pubmed PY - 2013/11/2/medline KW - 1-methyl-4-phenyl-1,2,3,6-tetrahydrodropyridine KW - 1-methyl-4-phenylpyridinium KW - 6-OHDA KW - 6-hydroxydopamine KW - AD KW - ALS KW - AMP-activated protein kinase KW - AMPK KW - Alzheimer’s disease KW - Amyotrophic Lateral Sclerosis KW - Amyotrophic lateral sclerosis KW - BAT KW - CK KW - Charcot-Marie-Tooth disease and Friedreich’s ataxia KW - Co-Q10 KW - CoQ10 KW - Creatine KW - Drp1 KW - Free radicals KW - HD KW - Huntington’s Disease KW - Huntington’s disease KW - KGDH KW - MFN KW - MMP KW - MPP+ KW - MPTP KW - Mitochondrial dysfunction KW - Neurodegenerative diseases KW - PCr KW - PD KW - PGC-1α KW - PPARs KW - Parkinson’s disease KW - ROS KW - Sirtuins KW - TAR DNA KW - TCA KW - TORC KW - Tfam KW - Transduceres of Creb-related binding protein KW - UCP-1 KW - alpha-ketoglutarate dehydrogenase KW - binding protein TDP-43 KW - brown adipose tissue KW - coenzymeQ10 KW - creatine kinase KW - dynamin-related protein 1 KW - mitochondrial DNA KW - mitochondrial membrane potential KW - mitochondrial transcription factor A KW - mitofusin KW - mtDNA KW - peroxisome proliferator-activated receptors KW - phosphocreatine KW - proliferator-activated receptor-gamma coactivator-1alpha KW - reactive oxygen species KW - tricarboxylic acid cycle KW - uncoupling proteins SP - 1 EP - 29 JF - Free radical biology & medicine JO - Free Radic Biol Med VL - 63 N2 - 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-1α, TORC-1, AMP kinase, Nrf2-ARE, and Sirtuins as novel therapeutic targets for neurodegenerative disorders. SN - 1873-4596 UR - https://www.unboundmedicine.com/medline/citation/23567191/Mitochondrial_diseases_of_the_brain_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0891-5849(13)00123-8 DB - PRIME DP - Unbound Medicine ER -