(Q36565597)

15 August 2017

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title

Mitochondria in amyotrophic lateral sclerosis: a trigger and a target (English)

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amyotrophic lateral sclerosis

15 August 2017

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author name string

Luc Dupuis

1

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15 August 2017

Jose-Luis Gonzalez de Aguilar

2

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15 August 2017

Hugues Oudart

3

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15 August 2017

Marc de Tapia

4

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15 August 2017

Luis Barbeito

5

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15 August 2017

Jean-Philippe Loeffler

6

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15 August 2017

1 January 2004

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Neurodegenerative Diseases

15 August 2017

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15 August 2017

1

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15 August 2017

6

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15 August 2017

245-254

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Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis

15 August 2017

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A gene encoding a putative GTPase regulator is mutated in familial amyotrophic lateral sclerosis 2

21 January 2018

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The gene encoding alsin, a protein with three guanine-nucleotide exchange factor domains, is mutated in a form of recessive amyotrophic lateral sclerosis

21 January 2018

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DNA/RNA helicase gene mutations in a form of juvenile amyotrophic lateral sclerosis (ALS4)

21 January 2018

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Senataxin, the ortholog of a yeast RNA helicase, is mutant in ataxia-ocular apraxia 2.

21 January 2018

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Alsin is a Rab5 and Rac1 guanine nucleotide exchange factor

21 January 2018

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Aggregation and Motor Neuron Toxicity of an ALS-Linked SOD1 Mutant Independent from Wild-Type SOD1

21 January 2018

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An adverse property of a familial ALS-linked SOD1 mutation causes motor neuron disease characterized by vacuolar degeneration of mitochondria

21 January 2018

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From Charcot to Lou Gehrig: deciphering selective motor neuron death in ALS

21 January 2018

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Wild-Type Nonneuronal Cells Extend Survival of SOD1 Mutant Motor Neurons in ALS Mice

21 January 2018

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Visualization of defective mitochondrial function in skeletal muscle fibers of patients with sporadic amyotrophic lateral sclerosis

21 January 2018

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Amyotrophic lateral sclerosis: oxidative energy metabolism and calcium homeostasis in peripheral blood lymphocytes.

21 January 2018

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Radical metabolism is partner to energy metabolism in mitochondria

21 January 2018

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Interactions between mitochondrial bioenergetics and cytoplasmic calcium in cultured cerebellar granule cells.

21 January 2018

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In situ respiration and bioenergetic status of mitochondria in primary cerebellar granule neuronal cultures exposed continuously to glutamate

21 January 2018

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Do defects in mitochondrial energy metabolism underlie the pathology of neurodegenerative diseases?

21 January 2018

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Acute decrease in net glutamate uptake during energy deprivation.

21 January 2018

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Charcot-Marie-Tooth disease neurofilament mutations disrupt neurofilament assembly and axonal transport.

21 January 2018

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Axonal mitochondrial transport and potential are correlated

21 January 2018

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Ultrastructural study of synapses in the anterior horn neurons of patients with amyotrophic lateral sclerosis

21 January 2018

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Early vacuolization and mitochondrial damage in motor neurons of FALS mice are not associated with apoptosis or with changes in cytochrome oxidase histochemical reactivity.

21 January 2018

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ALS-associated mutant SOD1G93A causes mitochondrial vacuolation by expansion of the intermembrane space and by involvement of SOD1 aggregation and peroxisomes

21 January 2018

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A quantitative histochemical assay for activities of mitochondrial electron transport chain complexes in mouse spinal cord sections.

21 January 2018

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Mitochondrial electron transport chain complex dysfunction in a transgenic mouse model for amyotrophic lateral sclerosis

21 January 2018

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Mutated human SOD1 causes dysfunction of oxidative phosphorylation in mitochondria of transgenic mice.

21 January 2018

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Selective changes in mitochondria respiratory properties in oxidative or glycolytic muscle fibers isolated from G93AhumanSOD1 transgenic mice

21 January 2018

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Deleted 4977-bp mitochondrial DNA mutation is associated with sporadic amyotrophic lateral sclerosis: a hospital-based case-control study.

21 January 2018

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Single-cell analysis of mtDNA deletion levels in sporadic amyotrophic lateral sclerosis

21 January 2018

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Mitochondrial DNA from platelets of sporadic ALS patients restores normal respiratory functions in rho(0) cells.

21 January 2018

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Mitochondrial respiratory chain function in skeletal muscle of ALS patients.

21 January 2018

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Cytochrome c oxidase subunit I microdeletion in a patient with motor neuron disease.

21 January 2018

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Mitochondriopathy as a differential diagnosis of amyotrophic lateral sclerosis

21 January 2018

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Mitochondriopathy mimicking amyotrophic lateral sclerosis

21 January 2018

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Programmed cell death in amyotrophic lateral sclerosis

21 January 2018

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Caspase-1 is activated in neural cells and tissue with amyotrophic lateral sclerosis-associated mutations in copper-zinc superoxide dismutase.

21 January 2018

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Caspase-1 and -3 are sequentially activated in motor neuron death in Cu,Zn superoxide dismutase-mediated familial amyotrophic lateral sclerosis

21 January 2018

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The crucial role of caspase-9 in the disease progression of a transgenic ALS mouse model

21 January 2018

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Minocycline inhibits cytochrome c release and delays progression of amyotrophic lateral sclerosis in mice

21 January 2018

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Bax and Bcl-2 interaction in a transgenic mouse model of familial amyotrophic lateral sclerosis

21 January 2018

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Factors controlling the uptake of yeast copper/zinc superoxide dismutase into mitochondria.

21 January 2018

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Subcellular distribution of superoxide dismutases (SOD) in rat liver: Cu,Zn-SOD in mitochondria

21 January 2018

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Toxicity of familial ALS-linked SOD1 mutants from selective recruitment to spinal mitochondria.

21 January 2018

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Nitric oxide, oxidants, and protein tyrosine nitration

21 January 2018

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Peroxynitrite reactions and formation in mitochondria

21 January 2018

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Motoneuron death triggered by a specific pathway downstream of Fas. potentiation by ALS-linked SOD1 mutations.

21 January 2018

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Mitochondrial respiratory chain-dependent generation of superoxide anion and its release into the intermembrane space

21 January 2018

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Protein tyrosine nitration in the mitochondria from diabetic mouse heart. Implications to dysfunctional mitochondria in diabetes

21 January 2018

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Mitochondrial tyrosine nitration precedes chronic allograft nephropathy

21 January 2018

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Increased mitochondrial antioxidative activity or decreased oxygen free radical propagation prevent mutant SOD1-mediated motor neuron cell death and increase amyotrophic lateral sclerosis-like transgenic mouse survival

21 January 2018

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Immunohistochemical study on the distribution of MnSOD in the central nervous system of the transgenic mice expressing a human Cu/Zn SOD mutation

21 January 2018

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Cytochrome c nitration by peroxynitrite.

21 January 2018

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Amyotrophic lateral sclerosis-associated SOD1 mutant proteins bind and aggregate with Bcl-2 in spinal cord mitochondria

21 January 2018

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Expression of a Cu,Zn superoxide dismutase typical of familial amyotrophic lateral sclerosis induces mitochondrial alteration and increase of cytosolic Ca2+ concentration in transfected neuroblastoma SH-SY5Y cells

21 January 2018

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Mitochondrial dysfunction due to mutant copper/zinc superoxide dismutase associated with amyotrophic lateral sclerosis is reversed by N-acetylcysteine.

21 January 2018

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Mitochondrial dysfunction in a cell culture model of familial amyotrophic lateral sclerosis

21 January 2018

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Evidence for defective energy homeostasis in amyotrophic lateral sclerosis: benefit of a high-energy diet in a transgenic mouse model

21 January 2018

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Nutritional assessment and survival in ALS patients.

21 January 2018

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Intrathecal cyclosporin prolongs survival of late-stage ALS mice

21 January 2018

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Coenzyme Q10 administration increases brain mitochondrial concentrations and exerts neuroprotective effects

21 January 2018

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Effects of an inhibitor of poly(ADP-ribose) polymerase, desmethylselegiline, trientine, and lipoic acid in transgenic ALS mice.

21 January 2018

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Neuroprotective effects of creatine in a transgenic animal model of amyotrophic lateral sclerosis.

21 January 2018

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A randomized sequential trial of creatine in amyotrophic lateral sclerosis

21 January 2018

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Additive neuroprotective effects of minocycline with creatine in a mouse model of ALS.

21 January 2018

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Inhibition of ICE slows ALS in mice

21 January 2018

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ALS, SOD and peroxynitrite

21 January 2018

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21 January 2018

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