Journal ArticleDOI
Mitochondria, free radicals, and neurodegeneration.
TLDR
Evidence implicating energy defects in neurodegenerative diseases comes from similarities to known mitochondrial disorders, including delayed and variable age of onset, slow progression, and symmetric degeneration of circumscribed groups of neurons.About:
This article is published in Current Opinion in Neurobiology.The article was published on 1996-10-01. It has received 443 citations till now. The article focuses on the topics: Neurodegeneration & Mitochondrial disease.read more
Citations
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Journal ArticleDOI
Mitochondrial metabolism of reactive oxygen species.
A. Yu. Andreyev,A. Yu. Andreyev,Yu. E. Kushnareva,Yu. E. Kushnareva,Anatoly A. Starkov,Anatoly A. Starkov +5 more
TL;DR: It is suggested that mitochondria augment intracellular oxidative stress due primarily to failure of their ROS removal systems, whereas the role of mitochondrial ROS emission is yet to be determined and a net increase in mitochondrial ROS production in situ remains to be demonstrated.
Journal ArticleDOI
Mitochondria and Neuronal Survival
TL;DR: The functional bioenergetics of isolated mitochondria are reviewed, with emphasis on the chemiosmotic proton circuit and the application (and occasional misapplication) of these principles to intact neurons.
Journal ArticleDOI
Oxidative stress and Alzheimer disease
TL;DR: Many free radical scavengers have produced promising results in relation to AD, as has desferrioxamine-an iron-chelating agent- and antiinflammatory drugs and estrogens, which also have an antioxidant effect.
Journal ArticleDOI
Complex III Releases Superoxide to Both Sides of the Inner Mitochondrial Membrane
TL;DR: It is demonstrated that Complex I-dependent superoxide is exclusively released into the matrix and that no detectable levels escape from intact mitochondria, fitting well with the proposed site of electron leak at Complex I.
Journal ArticleDOI
Lipofuscin: mechanisms of age-related accumulation and influence on cell function
Ulf T. Brunk,Alexei Terman +1 more
TL;DR: The accumulation of lipofuscin within postmitotic cells is a recognized hallmark of aging occuring with a rate inversely related to longevity as mentioned in this paper, whereas proliferative cells efficiently dilute it during division.
References
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Journal ArticleDOI
Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis
TL;DR: Tight genetic linkage between FALS and a gene that encodes a cytosolic, Cu/Zn-binding superoxide dismutase (SOD1), a homodimeric metalloenzyme that catalyzes the dismutation of the toxic superoxide anion O–2 to O2 and H2O2 is reported.
Journal ArticleDOI
Motor neuron degeneration in mice that express a human Cu,Zn superoxide dismutase mutation.
Mark E. Gurney,Haifeng Pu,Arlene Y. Chiu,Mauro C. Dal Canto,Cynthia Y. Polchow,Denise D. Alexander,Jan Caliendo,Afif Hentati,Young W. Kwon,Han Xiang Deng,W. Chen,Ping Zhai,Robert L. Sufit,Teepu Siddique +13 more
TL;DR: In this article, the authors found that mutations of human Cu,Zn superoxide dismutase (SOD) contribute to the pathogenesis of familial amyotrophic lateral sclerosis (ALS).
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Human cells lacking mtDNA: repopulation with exogenous mitochondria by complementation
Michael P. King,Giuseppe Attardi +1 more
TL;DR: Transformants obtained with various mitochondrial donors exhibited a respiratory phenotype that was in most cases distinct from that of the rho 0 parent or the donor, indicating that the genotypes of the mitochondrial and nuclear genomes as well as their specific interactions play a role in the respiratory competence of a cell.
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Peroxynitrite-mediated tyrosine nitration catalyzed by superoxide dismutase
Harry Ischiropoulos,Ling Zhu,Jun Chen,Michael Y. Tsai,James C. Martin,Craig D. Smith,Joseph S. Beckman +6 more
TL;DR: The mechanism appears to involve peroxynitrite initially reacting with the active site copper to form an intermediate with the reactivity of nitronium ion (NO2+), which then nitrates tyrosine on a second molecule of superoxide dismutase.
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An adverse property of a familial ALS-linked SOD1 mutation causes motor neuron disease characterized by vacuolar degeneration of mitochondria
Philip C. Wong,Carlos A. Pardo,David R. Borchelt,Michael K. Lee,Neal G. Copeland,Nancy A. Jenkins,Sangram S. Sisodia,Don W. Cleveland,Don W. Cleveland,Donald L. Price +9 more
TL;DR: Mutations in Cu/Zn superoxide dismutase cause a subset of cases of familial amyotrophic lateral sclerosis, and four lines of mice accumulating one of these mutant proteins (G37R) develop severe, progressive motor neuron disease.