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

Titel:	Mitochondrial diseases of the brain.
Autor/in / Beteiligte Person:	Chaturvedi, RK ; Flint Beal, M
Zeitschrift:	Free radical biology & medicine, Jg. 63 (2013-10-01), S. 1-29
Veröffentlichung:	Tarrytown, NY : Elsevier Science ; <i>Original Publication</i>: New York : Pergamon, c1987-, 2013
Medientyp:	academicJournal
ISSN:	1873-4596 (electronic)
DOI:	10.1016/j.freeradbiomed.2013.03.018
Schlagwort:	Alzheimer Disease metabolism Alzheimer Disease pathology Amyotrophic Lateral Sclerosis metabolism Amyotrophic Lateral Sclerosis pathology Brain pathology Charcot-Marie-Tooth Disease metabolism Charcot-Marie-Tooth Disease pathology Free Radicals toxicity Friedreich Ataxia metabolism Friedreich Ataxia pathology Humans Huntington Disease metabolism Huntington Disease pathology Mitochondria pathology Nerve Degeneration physiopathology Parkinson Disease metabolism Parkinson Disease pathology Brain metabolism Mitochondria metabolism Mitochondrial Diseases pathology Nerve Degeneration metabolism
Sonstiges:	Nachgewiesen in: MEDLINE Sprachen: English Publication Type: 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: English [Free Radic Biol Med] 2013 Oct; Vol. 63, pp. 1-29. <i>Date of Electronic Publication: </i>2013 Apr 06. MeSH Terms: Brain / metabolism ; Mitochondria / metabolism ; Mitochondrial Diseases / pathology ; Nerve Degeneration / metabolism ; Alzheimer Disease / metabolism ; Alzheimer Disease / pathology ; Amyotrophic Lateral Sclerosis / metabolism ; Amyotrophic Lateral Sclerosis / pathology ; Brain / pathology ; Charcot-Marie-Tooth Disease / metabolism ; Charcot-Marie-Tooth Disease / pathology ; Free Radicals / toxicity ; Friedreich Ataxia / metabolism ; Friedreich Ataxia / pathology ; Humans ; Huntington Disease / metabolism ; Huntington Disease / pathology ; Mitochondria / pathology ; Nerve Degeneration / physiopathology ; Parkinson Disease / metabolism ; Parkinson Disease / pathology Contributed Indexing: Keywords: 1-methyl-4-phenyl-1,2,3,6-tetrahydrodropyridine; 1-methyl-4-phenylpyridinium; 6-OHDA; 6-hydroxydopamine; AD; ALS; AMP-activated protein kinase; AMPK; Alzheimer’s disease; Amyotrophic Lateral Sclerosis; Amyotrophic lateral sclerosis; BAT; CK; Charcot-Marie-Tooth disease and Friedreich’s ataxia; Co-Q10; CoQ10; Creatine; Drp1; Free radicals; HD; Huntington’s Disease; Huntington’s disease; KGDH; MFN; MMP; MPP+; MPTP; Mitochondrial dysfunction; Neurodegenerative diseases; PCr; PD; PGC-1α; PPARs; Parkinson’s disease; ROS; Sirtuins; TAR DNA; TCA; TORC; Tfam; Transduceres of Creb-related binding protein; UCP-1; alpha-ketoglutarate dehydrogenase; binding protein TDP-43; brown adipose tissue; coenzymeQ10; creatine kinase; dynamin-related protein 1; mitochondrial DNA; mitochondrial membrane potential; mitochondrial transcription factor A; mitofusin; mtDNA; peroxisome proliferator-activated receptors; phosphocreatine; proliferator-activated receptor-gamma coactivator-1alpha; reactive oxygen species; tricarboxylic acid cycle; uncoupling proteins Substance Nomenclature: 0 (Free Radicals) Entry Date(s): Date Created: 20130410 Date Completed: 20131101 Latest Revision: 20220309 Update Code: 20240513

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Mitochondrial diseases of the brain.