Mitochondrial involvement in amyotrophic lateral sclerosis: trigger or target? (Q36444334)

14 August 2017

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title

Mitochondrial involvement in amyotrophic lateral sclerosis: trigger or target? (English)

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amyotrophic lateral sclerosis

14 August 2017

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author name string

Sandra R Bacman

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1

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14 August 2017

Walter G Bradley

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2

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14 August 2017

Carlos T Moraes

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3

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14 August 2017

1 April 2006

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14 August 2017

Molecular Neurobiology

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14 August 2017

33

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14 August 2017

2

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14 August 2017

113-131

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https://scigraph.springernature.com/pub.10.1385/mn:33:2:113

14 August 2017

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Accumulation of SOD1 mutants in postnatal motoneurons does not cause motoneuron pathology or motoneuron disease

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Neurofilaments and orthograde transport are reduced in ventral root axons of transgenic mice that express human SOD1 with a G93A mutation

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

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

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Caspase-12 cleavage and increased oxidative stress during motoneuron degeneration in transgenic mouse model of ALS.

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Ultrastructural changes of mitochondria in the skeletal muscle of patients with amyotrophic lateral sclerosis

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Glutamate receptors: RNA editing and death of motor neurons

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

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Increased mitochondrial superoxide dismutase activity in Parkinson's disease but not amyotrophic lateral sclerosis motor cortex.

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Mitochondrial localization of mutant superoxide dismutase 1 triggers caspase-dependent cell death in a cellular model of familial amyotrophic lateral sclerosis

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The roles of free radicals in amyotrophic lateral sclerosis: reactive oxygen species and elevated oxidation of protein, DNA, and membrane phospholipids

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

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Morphological evidence for lipid peroxidation and protein glycoxidation in spinal cords from sporadic amyotrophic lateral sclerosis patients

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Skeletal muscle properties in a transgenic mouse model for amyotrophic lateral sclerosis: effects of creatine treatment

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Mitochondrial fusion in human cells is efficient, requires the inner membrane potential, and is mediated by mitofusins.

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Protein aggregation in motor neurone disorders.

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Cytochrome c nitration by peroxynitrite.

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Genetic linkage analysis of familial amyotrophic lateral sclerosis using human chromosome 21 microsatellite DNA markers.

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12 December 2020

An ALS mouse model with a permeable blood-brain barrier benefits from systemic cyclosporine A treatment.

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Programmed cell death in amyotrophic lateral sclerosis: a mechanism of pathogenic and therapeutic importance

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12 December 2020

Functional Role of Caspase-1 and Caspase-3 in an ALS Transgenic Mouse Model

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12 December 2020

Reductions in acidic amino acids andN-acetylaspartylglutamate in amyotrophic lateral sclerosis CNS

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12 December 2020

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12 December 2020

Cytoskeletal defects in amyotrophic lateral sclerosis (motor neuron disease).

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12 December 2020

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

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12 December 2020

Mitochondrial enzyme activity in amyotrophic lateral sclerosis: implications for the role of mitochondria in neuronal cell death

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12 December 2020

ALS-linked Cu/Zn-SOD mutation increases vulnerability of motor neurons to excitotoxicity by a mechanism involving increased oxidative stress and perturbed calcium homeostasis

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12 December 2020

Mechanism and treatment of motoneuron degeneration in ALS: what have SOD1 mutants told us?

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12 December 2020

Ubiquitin-immunoreactive intraneuronal inclusions in amyotrophic lateral sclerosis. Morphology, distribution, and specificity.

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12 December 2020

The mitochondrial theory of aging: dead or alive?

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12 December 2020

A low expressor line of transgenic mice carrying a mutant human Cu,Zn superoxide dismutase (SOD1) gene develops pathological changes that most closely resemble those in human amyotrophic lateral sclerosis

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12 December 2020

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

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12 December 2020

Biomagnification of cyanobacterial neurotoxins and neurodegenerative disease among the Chamorro people of Guam

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12 December 2020

Reduction of oxidative stress in amyotrophic lateral sclerosis following pramipexole treatment

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12 December 2020

Cardiolipins and biomembrane function

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12 December 2020

ALS-linked SOD1 mutant G85R mediates damage to astrocytes and promotes rapidly progressive disease with SOD1-containing inclusions.

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12 December 2020

Vascular endothelial growth factor prolongs survival in a transgenic mouse model of ALS.

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12 December 2020

Histological evidence of redox system breakdown caused by superoxide dismutase 1 (SOD1) aggregation is common to SOD1-mutated motor neurons in humans and animal models

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12 December 2020

Neuronal death in amyotrophic lateral sclerosis is apoptosis: possible contribution of a programmed cell death mechanism

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12 December 2020

Calcium-permeable AMPA receptors promote misfolding of mutant SOD1 protein and development of amyotrophic lateral sclerosis in a transgenic mouse model

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12 December 2020

Overexpression of mutated Cu,Zn-SOD in neuroblastoma cells results in cytoskeletal change.

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12 December 2020

Copper,zinc superoxide dismutase is primarily a cytosolic protein in human cells

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12 December 2020

Molecular chaperones in cellular protein folding

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12 December 2020

Development of central nervous system pathology in a murine transgenic model of human amyotrophic lateral sclerosis

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12 December 2020

Superoxide dismutase activity, oxidative damage, and mitochondrial energy metabolism in familial and sporadic amyotrophic lateral sclerosis

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12 December 2020

Mitochondrial fusion protein mutated in CMT2A.

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12 December 2020

Amyotrophic lateral sclerosis: a proposed mechanism

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12 December 2020

A fraction of yeast Cu,Zn-superoxide dismutase and its metallochaperone, CCS, localize to the intermembrane space of mitochondria. A physiological role for SOD1 in guarding against mitochondrial oxidative damage.

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12 December 2020

Up-regulation of protein chaperones preserves viability of cells expressing toxic Cu/Zn-superoxide dismutase mutants associated with amyotrophic lateral sclerosis

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12 December 2020

Mitochondrial involvement in amyotrophic lateral sclerosis

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12 December 2020

Glutamate transporter EAAT2 splice variants occur not only in ALS, but also in AD and controls

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12 December 2020

Copper-zinc superoxide dismutase and amyotrophic lateral sclerosis

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12 December 2020

Polarographic assay and intracellular distribution of superoxide dismutase in rat liver

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12 December 2020

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

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12 December 2020

Massive mitochondrial degeneration in motor neurons triggers the onset of amyotrophic lateral sclerosis in mice expressing a mutant SOD1

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12 December 2020

Mitochondrial superoxide simutase. Site of synthesis and intramitochondrial localization

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12 December 2020

Could mitochondrial haplogroups play a role in sporadic amyotrophic lateral sclerosis?

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12 December 2020

Cytochrome c association with the inner mitochondrial membrane is impaired in the CNS of G93A-SOD1 mice.

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12 December 2020

Evidence that mitochondria buffer physiological Ca2+ loads in lizard motor nerve terminals

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12 December 2020

VEGF-induced activation of the PI3-K/Akt pathway reduces mutant SOD1-mediated motor neuron cell death.

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12 December 2020

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

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12 December 2020

Pivotal role of mitochondrial calcium uptake in neural cell apoptosis and necrosis

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12 December 2020

The cardiolipin-cytochrome c interaction and the mitochondrial regulation of apoptosis

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12 December 2020

Altered ionic conductances in axons of transgenic mouse expressing the human neurofilament heavy gene: A mouse model of amyotrophic lateral sclerosis

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12 December 2020

Mitochondrial phospholipid hydroperoxide glutathione peroxidase inhibits the release of cytochrome c from mitochondria by suppressing the peroxidation of cardiolipin in hypoglycaemia-induced apoptosis

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12 December 2020

Aberrant RNA processing in a neurodegenerative disease: the cause for absent EAAT2, a glutamate transporter, in amyotrophic lateral sclerosis

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12 December 2020

Initiation of apoptotic signal by the peroxidation of cardiolipin of mitochondria

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12 December 2020

The role of creatine in the management of amyotrophic lateral sclerosis and other neurodegenerative disorders

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12 December 2020

Mitochondrial DNA and respiratory chain function in spinal cords of ALS patients

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12 December 2020

Mitochondrial respiratory chain function in skeletal muscle of ALS patients.

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12 December 2020

Decrease of Hsp25 protein expression precedes degeneration of motoneurons in ALS-SOD1 mice

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12 December 2020

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

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12 December 2020

Cyclooxygenase 2 inhibition protects motor neurons and prolongs survival in a transgenic mouse model of ALS.

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12 December 2020

Impairment of fast axonal transport in the proximal axons of anterior horn neurons in amyotrophic lateral sclerosis

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12 December 2020

Abnormal excitatory amino acid metabolism in amyotrophic lateral sclerosis

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12 December 2020

CuZn superoxide dismutase (SOD1) accumulates in vacuolated mitochondria in transgenic mice expressing amyotrophic lateral sclerosis-linked SOD1 mutations

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12 December 2020

Delaying caspase activation by Bcl-2: A clue to disease retardation in a transgenic mouse model of amyotrophic lateral sclerosis

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12 December 2020

Aging, energy, and oxidative stress in neurodegenerative diseases.

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12 December 2020

Axonal transport of mitochondria along microtubules and F-actin in living vertebrate neurons

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12 December 2020

Metabolic dysfunction in familial, but not sporadic, amyotrophic lateral sclerosis.

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12 December 2020

Increased lipid peroxidation in sera of ALS patients: a potential biomarker of disease burden

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12 December 2020

Dysfunctional mitochondrial respiration in the wobbler mouse brain

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12 December 2020

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

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12 December 2020

Superoxide anion radical (O2-.), superoxide dismutases, and related matters

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12 December 2020

Aggregation of mutant Cu/Zn superoxide dismutase proteins in a culture model of ALS

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12 December 2020

The effect of reactive oxygen species generated from the mitochondrial electron transport chain on the cytochrome c oxidase activity and on the cardiolipin content in bovine heart submitochondrial particles

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12 December 2020

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

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12 December 2020

ALS-linked Cu/Zn-SOD mutation impairs cerebral synaptic glucose and glutamate transport and exacerbates ischemic brain injury

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12 December 2020

Mutant Cu, Zn superoxide dismutase that causes motoneuron degeneration is present in mitochondria in the CNS

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12 December 2020

A role for neurofilaments in the pathogenesis of amyotrophic lateral sclerosis

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12 December 2020

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

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12 December 2020

Cytoskeletal abnormalities in amyotrophic lateral sclerosis: beneficial or detrimental effects?

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12 December 2020

Oxidative damage and mutation to mitochondrial DNA and age-dependent decline of mitochondrial respiratory function

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12 December 2020

Inhibition of Chaperone Activity Is a Shared Property of Several Cu,Zn-Superoxide Dismutase Mutants That Cause Amyotrophic Lateral Sclerosis

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12 December 2020

No effect of creatine on respiratory distress in amyotrophic lateral sclerosis

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12 December 2020

Characterization of neuronal intermediate filament protein expression in cervical spinal motor neurons in sporadic amyotrophic lateral sclerosis (ALS).

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12 December 2020

Loss of cardiolipin and mitochondria during programmed neuronal death: evidence of a role for lipid peroxidation and autophagy

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12 December 2020

Loss of molecular interaction between cytochrome c and cardiolipin due to lipid peroxidation

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12 December 2020

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

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12 December 2020

Manganese superoxide dismutase levels are elevated in a proportion of amyotrophic lateral sclerosis patient cell lines

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12 December 2020

Brain glutamate deficiency in amyotrophic lateral sclerosis.

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12 December 2020

Cloned glutamate receptors

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12 December 2020

The RNA of the glutamate transporter EAAT2 is variably spliced in amyotrophic lateral sclerosis and normal individuals

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12 December 2020

Control of mitochondrial morphology by a human mitofusin

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12 December 2020

Neuropathological changes in two lines of mice carrying a transgene for mutant human Cu,Zn SOD, and in mice overexpressing wild type human SOD: a model of familial amyotrophic lateral sclerosis (FALS).

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12 December 2020

CuZnSOD and MnSOD immunoreactivity in brain stem motor neurons from amyotrophic lateral sclerosis patients.

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12 December 2020

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

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12 December 2020

Focal loss of the glutamate transporter EAAT2 in a transgenic rat model of SOD1 mutant-mediated amyotrophic lateral sclerosis (ALS).

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12 December 2020

Is ALS caused by an altered oxidative activity of mutant superoxide dismutase?

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12 December 2020

Copper chaperone for superoxide dismutase is essential to activate mammalian Cu/Zn superoxide dismutase

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12 December 2020

Superoxide dismutases

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12 December 2020

Hsp70 and Hsp40 improve neurite outgrowth and suppress intracytoplasmic aggregate formation in cultured neuronal cells expressing mutant SOD1.

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12 December 2020

Restricted expression of G86R Cu/Zn superoxide dismutase in astrocytes results in astrocytosis but does not cause motoneuron degeneration

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12 December 2020

Formation of high molecular weight complexes of mutant Cu, Zn-superoxide dismutase in a mouse model for familial amyotrophic lateral sclerosis.

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12 December 2020

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

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12 December 2020

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

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12 December 2020

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

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12 December 2020

Parvalbumin and calbindin D-28k in the human motor system and in motor neuron disease.

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12 December 2020

Ultrastructural evidence for altered calcium in motor nerve terminals in amyotropic lateral sclerosis

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12 December 2020

Mitochondria in sporadic amyotrophic lateral sclerosis

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12 December 2020

The copper chaperone for superoxide dismutase

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12 December 2020

The Charcot-Marie-Tooth type 2A gene product, Mfn2, up-regulates fuel oxidation through expression of OXPHOS system

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12 December 2020

Stimulation‐Induced Mitochondrial [Ca2+] Elevations in Mouse Motor Terminals: Comparison of Wild‐Type with SOD1‐G93A

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12 December 2020

Diagnosis of amyotrophic lateral sclerosis

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12 December 2020

Mutant SOD1 linked to familial amyotrophic lateral sclerosis, but not wild-type SOD1, induces ER stress in COS7 cells and transgenic mice.

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12 December 2020

Relationship of oxygen radical-induced lipid peroxidative damage to disease onset and progression in a transgenic model of familial ALS.

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12 December 2020

Mitochondrial DNA abnormalities in skeletal muscle of patients with sporadic amyotrophic lateral sclerosis

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12 December 2020

Recruitment of the mitochondrial-dependent apoptotic pathway in amyotrophic lateral sclerosis

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12 December 2020

Mitochondrial DNA deletion mutation levels are elevated in ALS brains

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12 December 2020

A clinical trial of creatine in ALS.

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12 December 2020

Identifiers

DOI

10.1385/MN:33:2:113

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Dimensions Publication ID

14 August 2017

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