Mitochondrial Diseases of the Brain

被引：347
作者：
Chaturvedi, Rajnish K. [1 ]
Beal, M. Flint [2 ]
机构：
[1] CSIR IITR, Lucknow 226001, Uttar Pradesh, India
[2] Cornell Univ, Weill Med Coll, Dept Neurol & Neurosci, New York, NY 10065 USA
来源：
FREE RADICAL BIOLOGY AND MEDICINE | 2013年 / 63卷
关键词：
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; AMYOTROPHIC-LATERAL-SCLEROSIS; TRANSGENIC MOUSE MODEL; CYTOCHROME-C-OXIDASE; AMYLOID PRECURSOR PROTEIN; COMPLEX-I DEFICIENCY; TARGETED ANTIOXIDANT MITOQ; NEURONAL CELL-DEATH; KETOGLUTARATE DEHYDROGENASE COMPLEX; PERMEABLE PEPTIDE ANTIOXIDANTS; WATER-SOLUBLE FORMULATION;
D O I：
10.1016/j.freeradbiomed.2013.03.018
中图分类号：
Q5 [生物化学]; Q7 [分子生物学];
学科分类号：
071010 ; 081704 ;
摘要：
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.
引用
收藏
页码：1 / 29
页数：29
相关论文
共 488 条
[1]   Mitochondrial translation initiation factor 3 gene polymorphism associated with Parkinson's disease [J].
Abahuni, Nadine ;
Gispert, Suzana ;
Bauer, Peter ;
Riess, Olaf ;
Krueger, Rejko ;
Becker, Tim ;
Auburger, Georg .
NEUROSCIENCE LETTERS, 2007, 414 (02) :126-129
[2]   Expanding insights of mitochondrial dysfunction in Parkinson's disease [J].
Abou-Sleiman, PM ;
Muqit, MMK ;
Wood, NW .
NATURE REVIEWS NEUROSCIENCE, 2006, 7 (03) :207-219
[3]   Mitochondrial DNA damage is a hallmark of chemically induced and the R6/2 transgenic model of Huntington's disease [J].
Acevedo-Torres, Karina ;
Berrios, Lexsy ;
Rosario, Nydia ;
Dufault, Vanessa ;
Skatchkov, Serguei ;
Eaton, Misty J. ;
Torres-Ramos, Carlos A. ;
Ayala-Torres, Sylvette .
DNA REPAIR, 2009, 8 (01) :126-136
[4]   Creatine and Its Potential Therapeutic Value for Targeting Cellular Energy Impairment in Neurodegenerative Diseases [J].
Adhihetty, Peter J. ;
Beal, M. Flint .
NEUROMOLECULAR MEDICINE, 2008, 10 (04) :275-290
[5]   Targeting an antioxidant to mitochondria decreases cardiac ischemia-reperfusion injury [J].
Adlam, VJ ;
Harrison, JC ;
Porteous, CM ;
James, AM ;
Smith, RAJ ;
Murphy, MP ;
Sammut, IA .
FASEB JOURNAL, 2005, 19 (09) :1088-1095
[6]   α-Synuclein gene duplication is present in sporadic Parkinson disease [J].
Ahn, T. -B. ;
Kim, S. Y. ;
Kim, J. Y. ;
Park, S. -S. ;
Lee, D. S. ;
Min, H. J. ;
Kim, Y. K. ;
Kim, S. E. ;
Kim, J. -M. ;
Kim, H. -J. ;
Cho, J. ;
Jeon, B. S. .
NEUROLOGY, 2008, 70 (01) :43-49
[7]   The expression of several mitochondrial and nuclear genes encoding the subunits of electron transport chain enzyme complexes, cytochrome c oxidase, and NADH dehydrogenase, in different brain regions in Alzheimer's disease [J].
Aksenov, MY ;
Tucker, HM ;
Nair, P ;
Aksenova, MV ;
Butterfield, DA ;
Estus, S ;
Markesbery, WR .
NEUROCHEMICAL RESEARCH, 1999, 24 (06) :767-774
[8]   DJ-1 protects the nigrostriatal axis from the neurotoxin MPTP by modulation of the AKT pathway [J].
Aleyasin, Hossein ;
Rousseaux, Maxime W. C. ;
Marcogliese, Paul C. ;
Hewitt, Sarah J. ;
Irrcher, Isabella ;
Joselin, Alvin P. ;
Parsanejad, Mohammad ;
Kim, Raymond H. ;
Rizzu, Patrizia ;
Callaghan, Steve M. ;
Slack, Ruth S. ;
Mak, Tak W. ;
Park, David S. .
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 2010, 107 (07) :3186-3191
[9]   Evidence of apoptosis and mitochondrial abnormalities in peripheral blood cells of Huntington's disease patients [J].
Almeida, Sandra ;
Sarmento-Ribeiro, Ana Bela ;
Januario, Cristina ;
Rego, A. Cristina ;
Oliveira, Catarina R. .
BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS, 2008, 374 (04) :599-603
[10]   Mitochondrial fusion and function in Charcot-Marie-Tooth type 2A patient fibroblasts with mitofusin 2 mutations [J].
Amiott, Elizabeth A. ;
Lott, Paul ;
Soto, Jamie ;
Kang, Peter B. ;
McCaffery, J. Michael ;
DiMauro, Salvatore ;
Abel, E. Dale ;
Flanigan, Kevin M. ;
Lawson, Victoria H. ;
Shaw, Janet M. .
EXPERIMENTAL NEUROLOGY, 2008, 211 (01) :115-127
12345678910