Oxidative Stress and Altered Mitochondrial Function in Neurodegenerative Diseases: Lessons From Mouse Models

Suppression of basal autophagy in neural cells causes neurodegenerative disease in mice

2006 • 3,685 citations

Parkin is recruited selectively to impaired mitochondria and promotes their autophagy

2008 • 3,605 citations

Loss of autophagy in the central nervous system causes neurodegeneration in mice

2006 • 3,331 citations

Pathways towards and away from Alzheimer's disease

2004 • 2,936 citations

Fission and selective fusion govern mitochondrial segregation and elimination by autophagy

2008 • 2,822 citations

Physiological functions of thioredoxin and thioredoxin reductase

2000 • 2,440 citations

Suppression of Reactive Oxygen Species and Neurodegeneration by the PGC-1 Transcriptional Coactivators

2006 • 2,113 citations

Oxidative damage and mitochondrial decay in aging.

1994 • 2,024 citations

Oxidative Damage Is the Earliest Event in Alzheimer Disease

2001 • 1,833 citations

Dynamin-related Protein Drp1 Is Required for Mitochondrial Division in Mammalian Cells

2001 • 1,739 citations

High protonic potential actuates a mechanism of production of reactive oxygen species in mitochondria

1997 • 1,695 citations

Redox Regulation of Cell Survival

2008 • 1,645 citations

Cyclophilin D-dependent mitochondrial permeability transition regulates some necrotic but not apoptotic cell death

2005 • 1,570 citations

Mutations in the mitochondrial GTPase mitofusin 2 cause Charcot-Marie-Tooth neuropathy type 2A

2004 • 1,505 citations

Autophagy: molecular machinery for self-eating

2005 • 1,468 citations

Extensive Involvement of Autophagy in Alzheimer Disease: An Immuno-Electron Microscopy Study

2005 • 1,435 citations

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

1995 • 1,424 citations

The Importance of Dendritic Mitochondria in the Morphogenesis and Plasticity of Spines and Synapses

2004 • 1,400 citations

Mitochondrial dynamics-fusion, fission, movement, and mitophagy-in neurodegenerative diseases

2009 • 1,363 citations

Excess brain protein oxidation and enzyme dysfunction in normal aging and in Alzheimer disease.

1991 • 1,290 citations

OPA1, encoding a dynamin-related GTPase, is mutated in autosomal dominant optic atrophy linked to chromosome 3q28

2000 • 1,269 citations

Mitochondrial Oxidative Stress: Implications for Cell Death

2006 • 1,212 citations

Generation of reactive oxygen species by the mitochondrial electron transport chain

2002 • 1,208 citations

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

1998 • 1,207 citations

Mitochondria and Neuronal Survival

2000 • 1,206 citations

OPA1 requires mitofusin 1 to promote mitochondrial fusion

2004 • 1,158 citations

Mice Deficient in Cellular Glutathione Peroxidase Show Increased Vulnerability to Malonate, 3-Nitropropionic Acid, and 1-Methyl-4-Phenyl-1,2,5,6-Tetrahydropyridine

2000 • 1,112 citations

Aggregate-prone proteins with polyglutamine and polyalanine expansions are degraded by autophagy

2002 • 1,076 citations

Involvement of oxidative stress-induced abnormalities in ceramide and cholesterol metabolism in brain aging and Alzheimer's disease

2004 • 1,063 citations

The ADP/ATP translocator is not essential for the mitochondrial permeability transition pore

2004 • 1,045 citations

FoxOs Are Lineage-Restricted Redundant Tumor Suppressors and Regulate Endothelial Cell Homeostasis

2007 • 1,018 citations

Apoptosis and autophagy in nigral neurons of patients with Parkinson's disease.

1997 • 1,016 citations

Early mitochondrial calcium defects in Huntington's disease are a direct effect of polyglutamines

2002 • 1,009 citations

Mechanism of Toxicity in Rotenone Models of Parkinson's Disease

2003 • 1,003 citations

Immunohistochemical detection of 4-hydroxynonenal protein adducts in Parkinson disease.

1996 • 976 citations

Mitochondria in Neuroplasticity and Neurological Disorders

2008 • 964 citations

Mitochondrial Dysfunction and Oxidative Damage in parkin-deficient Mice

2004 • 959 citations

The role of alterations in membrane lipid composition in enabling physiological adaptation of organisms to their physical environment

1990 • 950 citations

Voltage-dependent anion channels are dispensable for mitochondrial-dependent cell death

2007 • 895 citations

Loss of PINK1 Function Promotes Mitophagy through Effects on Oxidative Stress and Mitochondrial Fission

2009 • 891 citations

Mitochondrial Glutathione, a Key Survival Antioxidant

2009 • 880 citations

Cyclophilin D deficiency attenuates mitochondrial and neuronal perturbation and ameliorates learning and memory in Alzheimer's disease

2008 • 874 citations

Mitochondrial bioenergetic deficit precedes Alzheimer's pathology in female mouse model of Alzheimer's disease

2009 • 865 citations

Mitochondrial Fusion Protects against Neurodegeneration in the Cerebellum

2007 • 863 citations

A Double-Blind, Delayed-Start Trial of Rasagiline in Parkinson's Disease

2009 • 858 citations

The PINK1/Parkin pathway regulates mitochondrial morphology

2008 • 845 citations

Mitochondrial permeability transition and oxidative stress

2001 • 842 citations

Oxidative stress in brain agingImplications for therapeutics of neurodegenerative diseases

2002 • 810 citations

VDAC2 Inhibits BAK Activation and Mitochondrial Apoptosis

2003 • 789 citations

The Mitochondrial Protein hFis1 Regulates Mitochondrial Fission in Mammalian Cells through an Interaction with the Dynamin-Like Protein DLP1

2003 • 778 citations

Regulation of mitochondrial ATP synthesis by calcium: Evidence for a long-term metabolic priming

1999 • 776 citations

A role for uncoupling protein‐2 as a regulator of mitochondrial hydrogen peroxide generation

1997 • 756 citations

The selenoprotein GPX4 is essential for mouse development and protects from radiation and oxidative damage insults

2003 • 722 citations

Massive Mitochondrial Degeneration in Motor Neurons Triggers the Onset of Amyotrophic Lateral Sclerosis in Mice Expressing a Mutant SOD1

1998 • 706 citations

Mitofusin 1 and 2 play distinct roles in mitochondrial fusion reactions via GTPase activity

2004 • 662 citations

Loss of PINK1 causes mitochondrial functional defects and increased sensitivity to oxidative stress

2008 • 654 citations

PINK1-Associated Parkinson's Disease Is Caused by Neuronal Vulnerability to Calcium-Induced Cell Death

2009 • 640 citations

The mitochondrial permeability transition initiates autophagy in rat hepatocytes

2001 • 596 citations

Mutated Human SOD1 Causes Dysfunction of Oxidative Phosphorylation in Mitochondria of Transgenic Mice

2002 • 596 citations

Oxidative Stress, Thiol Reagents, and Membrane Potential Modulate the Mitochondrial Permeability Transition by Affecting Nucleotide Binding to the Adenine Nucleotide Translocase

1997 • 588 citations

Progressive parkinsonism in mice with respiratory-chain-deficient dopamine neurons

2007 • 561 citations

Monoamine oxidase inactivation: From pathophysiology to therapeutics

2008 • 560 citations

Nrf2-mediated neuroprotection in the MPTP mouse model of Parkinson's disease: Critical role for the astrocyte

2009 • 560 citations

Pink1 regulates mitochondrial dynamics through interaction with the fission/fusion machinery

2008 • 524 citations

The antioxidant role of coenzyme Q

2007 • 511 citations

Uncoupling protein-2 prevents neuronal death and diminishes brain dysfunction after stroke and brain trauma

2003 • 506 citations

Amyotrophic Lateral Sclerosis-Associated SOD1 Mutant Proteins Bind and Aggregate with Bcl-2 in Spinal Cord Mitochondria

2004 • 490 citations

p53 Mediates Cellular Dysfunction and Behavioral Abnormalities in Huntington’s Disease

2005 • 477 citations

The mitochondrial protease HtrA2 is regulated by Parkinson's disease-associated kinase PINK1

2007 • 467 citations

A gain-of-function of an amyotrophic lateral sclerosis-associated Cu,Zn-superoxide dismutase mutant: An enhancement of free radical formation due to a decrease in Km for hydrogen peroxide.

1996 • 464 citations

Dynein mutations impair autophagic clearance of aggregate-prone proteins

2005 • 442 citations

Nrf2 Activation in Astrocytes Protects against Neurodegeneration in Mouse Models of Familial Amyotrophic Lateral Sclerosis

2008 • 441 citations

The Absence of Mitochondrial Thioredoxin 2 Causes Massive Apoptosis, Exencephaly, and Early Embryonic Lethality in Homozygous Mice

2003 • 437 citations

Regulation of Intracellular Accumulation of Mutant Huntingtin by Beclin 1

2006 • 437 citations

Mitochondrial dysfunction: An early event in Alzheimer pathology accumulates with age in AD transgenic mice

2008 • 433 citations

Familial amyotrophic lateral sclerosis-linked SOD1 mutants perturb fast axonal transport to reduce axonal mitochondria content

2007 • 410 citations

Preventing Mitochondrial Fission Impairs Mitochondrial Function and Leads to Loss of Mitochondrial DNA

2008 • 395 citations

Crosstalk signaling between mitochondrial Ca2+ and ROS

2009 • 393 citations

Complete Dissociation of Motor Neuron Death from Motor Dysfunction by Bax Deletion in a Mouse Model of ALS

2006 • 382 citations

Characterization of Mammalian Sulfiredoxin and Its Reactivation of Hyperoxidized Peroxiredoxin through Reduction of Cysteine Sulfinic Acid in the Active Site to Cysteine

2004 • 375 citations

Integrating cytosolic calcium signals into mitochondrial metabolic responses

1998 • 373 citations

Loss of Parkin or PINK1 Function Increases Drp1-dependent Mitochondrial Fragmentation

2009 • 364 citations

Selective glutathione depletion of mitochondria by ethanol sensitizes hepatocytes to tumor necrosis factor

1998 • 361 citations

Parkinson Phenotype in Aged PINK1-Deficient Mice Is Accompanied by Progressive Mitochondrial Dysfunction in Absence of Neurodegeneration

2009 • 357 citations

Reactive oxygen species affect mitochondrial electron transport complex I activity through oxidative cardiolipin damage

2002 • 353 citations

Properties of the endosomal-lysosomal system in the human central nervous system: disturbances mark most neurons in populations at risk to degenerate in Alzheimer's disease

1996 • 346 citations

Cloning and Expression of a Novel Human Glutaredoxin (Grx2) with Mitochondrial and Nuclear Isoforms

2001 • 326 citations

Brain oxidative stress in a triple-transgenic mouse model of Alzheimer disease

2008 • 326 citations

Aconitase Couples Metabolic Regulation to Mitochondrial DNA Maintenance

2005 • 313 citations

The insulin paradox: aging, proteotoxicity and neurodegeneration

2008 • 297 citations

Mitochondria, calcium and cell death: A deadly triad in neurodegeneration

2009 • 294 citations

Regulation of Neuronal Survival Factor MEF2D by Chaperone-Mediated Autophagy

2009 • 294 citations

Coenzyme Q10 Prevents Apoptosis by Inhibiting Mitochondrial Depolarization Independently of Its Free Radical Scavenging Property

2003 • 293 citations

Increased plaque burden in brains of APP mutant MnSOD heterozygous knockout mice

2004 • 274 citations

GM1-Ganglioside Accumulation at the Mitochondria-Associated ER Membranes Links ER Stress to Ca2+-Dependent Mitochondrial Apoptosis

2009 • 273 citations

Selective association of misfolded ALS-linked mutant SOD1 with the cytoplasmic face of mitochondria

2008 • 264 citations

MAO-B Elevation in Mouse Brain Astrocytes Results in Parkinson's Pathology

2008 • 255 citations

Increased activity of brain and platelet monoamine oxidase in dementia of Alzheimer type

1980 • 254 citations

Inhibition of mitochondrial NADH dehydrogenase by pyridine derivatives and its possible relation to experimental and idiopathic parkinsonism

1986 • 250 citations

Familial ALS-superoxide dismutases associate with mitochondria and shift their redox potentials

2006 • 250 citations

Reduction in Mitochondrial Superoxide Dismutase Modulates Alzheimer's Disease-Like Pathology and Accelerates the Onset of Behavioral Changes in Human Amyloid Precursor Protein Transgenic Mice

2006 • 248 citations

Recruitment of mitochondrial cyclophilin to the mitochondrial inner membrane under conditions of oxidative stress that enhance the opening of a calcium-sensitive non-specific channel

1994 • 245 citations

Mutant Superoxide Dismutase 1 Forms Aggregates in the Brain Mitochondrial Matrix of Amyotrophic Lateral Sclerosis Mice

2005 • 242 citations

Mitochondrial Ca2+ as a key regulator of cell life and death

2007 • 237 citations

Mitochondrial superoxide and hydrogen peroxide generation, protein oxidative damage, and longevity in different species of flies

1995 • 234 citations

Identification and Functional Characterization of a Novel Mitochondrial Thioredoxin System in Saccharomyces cerevisiae

1999 • 223 citations

Phase II trial of CoQ10 for ALS finds insufficient evidence to justify phase III

2009 • 223 citations

Parkin-induced mitophagy in the pathogenesis of Parkinson disease

2009 • 223 citations

The Metabolism of Tyramine by Monoamine Oxidase A/B Causes Oxidative Damage to Mitochondrial DNA

1996 • 218 citations

Mitochondrial Glutathione: Importance and Transport

1998 • 218 citations

A fourth ADP/ATP carrier isoform in man: identification, bacterial expression, functional characterization and tissue distribution

2004 • 216 citations

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

2001 • 214 citations

Histone deacetylase inhibitors as therapeutics for polyglutamine disorders

2006 • 214 citations

Reduction of oxidative stress, amyloid deposition, and memory deficit by manganese superoxide dismutase overexpression in a transgenic mouse model of Alzheimer's disease

2009 • 213 citations

Memantine Improves Cognition and Reduces Alzheimer’s-Like Neuropathology in Transgenic Mice

2009 • 207 citations

Oxidative damage to mitochondrial DNA in Huntington's disease parietal cortex

1999 • 207 citations

Immunocytochemical Evidence for a Mitochondrially Located Nitric Oxide Synthase in Brain and Liver

1995 • 204 citations

Direct interaction of GD3 ganglioside with mitochondria generates reactive oxygen species followed by mitochondrial permeability transition, cytochrome c release, and caspase activation

2000 • 199 citations

Huntington’s disease: degradation of mutant huntingtin by autophagy

2008 • 197 citations

Randomized, Double-blind, Placebo-Controlled Trial on Symptomatic Effects of Coenzyme Q10 in Parkinson Disease

2007 • 195 citations

Induction of hyperphosphorylated tau in primary rat cortical neuron cultures mediated by oxidative stress and glycogen synthase kinase-3

2005 • 191 citations

Calcium Homeostasis and Mitochondrial Dysfunction in Striatal Neurons of Huntington Disease

2007 • 188 citations

Impaired autophagy: A mechanism of mitochondrial dysfunction in anoxic rat hepatocytes

2008 • 188 citations

Obesity and vulnerability of the CNS

2008 • 187 citations

Redox Control of Liver Function in Health and Disease

2009 • 183 citations

The mitochondrial permeability transition pore in motor neurons: Involvement in the pathobiology of ALS mice

2009 • 174 citations

Acetaldehyde impairs mitochondrial glutathione transport in HepG2 cells through endoplasmic reticulum stress

2003 • 166 citations

Mitochondrial dysfunction triggered by loss of HtrA2 results in the activation of a brain-specific transcriptional stress response

2008 • 165 citations

Thiamine-dependent enzyme changes in temporal cortex of patients with Alzheimer's disease

1990 • 160 citations

Insulin resistance and neurodegeneration: roles of obesity, type 2 diabetes mellitus and non-alcoholic steatohepatitis.

2009 • 154 citations

Mutant SOD1 in neuronal mitochondria causes toxicity and mitochondrial dynamics abnormalities

2009 • 152 citations

Tolerance of high-dose (3,000 mg/day) coenzyme Q10 in ALS

2005 • 149 citations

Sensitivity of the 2-oxoglutarate carrier to alcohol intake contributes to mitochondrial glutathione depletion

2003 • 146 citations

Increased Autophagic Degradation of Mitochondria in Alzheimer Disease

2007 • 146 citations

Structural basis for recruitment of mitochondrial fission complexes by Fis1

2007 • 135 citations

Overexpression of Superoxide Dismutase or Glutathione Peroxidase Protects against the Paraquat + Maneb-induced Parkinson Disease Phenotype

2005 • 134 citations

Oxidative Inactivation of Mitochondrial Aconitase Results in Iron and H2O2-Mediated Neurotoxicity in Rat Primary Mesencephalic Cultures

2009 • 132 citations

Proteomic and functional alterations in brain mitochondria from Tg2576 mice occur before amyloid plaque deposition

2007 • 130 citations

Inactivation of the 2‐oxo acid dehydrogenase complexes upon generation of intrinsic radical species

2002 • 130 citations

Postmortem changes in mitochondrial respiratory enzymes in brain and a preliminary observation in Parkinson's disease

1990 • 127 citations

Mitochondrial Function, Morphology, and Axonal Transport in Amyotrophic Lateral Sclerosis

2009 • 122 citations

Molecular biology of glutathione peroxidase 4: from genomic structure to developmental expression and neural function

2007 • 120 citations

Therapeutic role of sirtuins in neurodegenerative disease

2008 • 119 citations

Mice with a Partial Deficiency of Manganese Superoxide Dismutase Show Increased Vulnerability to the Mitochondrial Toxins Malonate, 3-Nitropropionic Acid, and MPTP

2001 • 114 citations

Early and persistent alterations in prefrontal cortex MAO A and B in Alzheimer's disease

2003 • 105 citations

Mice deficient in dihydrolipoamide dehydrogenase show increased vulnerability to MPTP, malonate and 3‐nitropropionic acid neurotoxicity

2004 • 104 citations

Critical Role of Mitochondrial Glutathione in the Survival of Hepatocytes during Hypoxia

2004 • 101 citations

Manganese Superoxide Dismutase Protects against 6-Hydroxydopamine Injury in Mouse Brains

2005 • 100 citations

Mitochondria and calcium flux as targets of neuroprotection caused by minocycline in cerebellar granule cells

2009 • 99 citations

Iron‐sulfur enzyme mediated mitochondrial superoxide toxicity in experimental Parkinson's disease

2004 • 91 citations

A high-throughput screening for mammalian cell death effectors identifies the mitochondrial phosphate carrier as a regulator of cytochrome c release

2007 • 90 citations

Chaperone-Mediated Autophagy

2008 • 89 citations

Attenuation of 6-hydroxydopamine-induced dopaminergic nigrostriatal lesions in superoxide dismutase transgenic mice

1998 • 87 citations

Cholesterol as a Risk Factor for Dementia and Cognitive Decline: A Systematic Review of Prospective Studies With Meta-Analysis

2008 • 81 citations

Selective degradation of mitochondria by mitophagy

2007 • 72 citations

Mitochondrial dihydrolipoyl succinyltransferase deficiency accelerates amyloid pathology and memory deficit in a transgenic mouse model of amyloid deposition

2009 • 67 citations

Parkin stabilizes PINK1 through direct interaction

2009 • 67 citations

S-nitrosothiol inhibition of mitochondrial complex I causes a reversible increase in mitochondrial hydrogen peroxide production

2006 • 63 citations

Water-soluble formulation of Coenzyme Q10 inhibits Bax-induced destabilization of mitochondria in mammalian cells

2006 • 63 citations

Initiation of Apoptotic Signal by the Peroxidation of Cardiolipin of Mitochondria

2004 • 58 citations

Axonal Mitochondrial Clusters Containing Mutant SOD1 in Transgenic Models of ALS

2009 • 55 citations

Absence of Nitric-oxide Synthase in Sequentially Purified Rat Liver Mitochondria

2009 • 52 citations

Lipid Trafficking Defects Increase Beclin-1 and Activate Autophagy in Niemann-Pick Type C Disease

2007 • 46 citations

Autophagic-Lysosomal Dysfunction and Neurodegeneration in Niemann-Pick Type C Mice: Lipid Starvation or Indigestion?

2007 • 37 citations

[14] Glutaredoxins and oxidative stress defense in yeast

2002 • 22 citations