Up‐regulation of mitochondrial uncoupling protein 3 reveals an early muscular metabolic defect in amyotrophic lateral sclerosis

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

1993 • 6,762 citations

Motor Neuron Degeneration in Mice that Express a Human Cu,Zn Superoxide Dismutase Mutation

1994 • 3,959 citations

El escorial World Federation of Neurology criteria for the diagnosis of amyotrophic lateral sclerosis

1994 • 2,090 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

Superoxide activates mitochondrial uncoupling proteins

2002 • 1,364 citations

Disruption of the uncoupling protein-2 gene in mice reveals a role in immunity and reactive oxygen species production

2000 • 1,068 citations

Uncoupling Protein-2 Negatively Regulates Insulin Secretion and Is a Major Link between Obesity, β Cell Dysfunction, and Type 2 Diabetes

2001 • 916 citations

The uncoupling protein homologues: UCP1, UCP2, UCP3, StUCP and AtUCP

2000 • 770 citations

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

1998 • 706 citations

Transgenic mice expressing an altered murine superoxide dismutase gene provide an animal model of amyotrophic lateral sclerosis.

1995 • 694 citations

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

1999 • 680 citations

Energy Metabolism in Uncoupling Protein 3 Gene Knockout Mice

2000 • 679 citations

Superoxide Dismutase Activity, Oxidative Damage, and Mitochondrial Energy Metabolism in Familial and Sporadic Amyotrophic Lateral Sclerosis

1993 • 627 citations

Mice overexpressing human uncoupling protein-3 in skeletal muscle are hyperphagic and lean

2000 • 615 citations

Relationship between configuration, function, and permeability in calcium-treated mitochondria.

1976 • 610 citations

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

2002 • 596 citations

Aging and acute exercise enhance free radical generation in rat skeletal muscle

1999 • 454 citations

Superoxide Dismutases

1986 • 450 citations

Familial adult motor neuron disease: amyotrophic lateralsclerosis

1986 • 401 citations

Lack of Obesity and Normal Response to Fasting and Thyroid Hormone in Mice Lacking Uncoupling Protein-3

2000 • 391 citations

Superoxide Activates Mitochondrial Uncoupling Protein 2 from the Matrix Side

2002 • 386 citations

Protein modification by the lipid peroxidation product 4‐hydroxynonenal in the spinal cords of amyotrophic lateral sclerosis patients

1998 • 382 citations

Mitochondrial Adaptations to Obesity-Related Oxidant Stress

2000 • 362 citations

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

2002 • 349 citations

Factors correlated with hypermetabolism in patients with amyotrophic lateral sclerosis

2001 • 338 citations

Increased oxidative damage to DNA in ALS patients

2000 • 316 citations

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

2001 • 273 citations

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

2000 • 252 citations

Increased 3‐nitrotyrosine and oxidative damage in mice with a human copper/zinc superoxide dismutase mutation

1997 • 248 citations

Mutant Cu, Zn Superoxide Dismutase that Causes Motoneuron Degeneration Is Present in Mitochondria in the CNS

2002 • 240 citations

UCP2 and UCP3 rise in starved rat skeletal muscle but mitochondrial proton conductance is unchanged

1999 • 235 citations

Familial amyotrophic lateral sclerosis

2002 • 231 citations

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

1997 • 221 citations

Mitochondria in Sporadic Amyotrophic Lateral Sclerosis

1998 • 179 citations

A significant portion of mitochondrial proton leak in intact thymocytes depends on expression of UCP2

2001 • 172 citations

Fast to slow transformation of denervated and electrically stimulated rat muscle

1998 • 163 citations

Nogo Provides a Molecular Marker for Diagnosis of Amyotrophic Lateral Sclerosis

2002 • 163 citations

Generation of superoxide anion by mitochondria and impairment of their functions during anoxia and reoxygenation in vitro

1998 • 145 citations

Uncoupling Protein-2 Participates in Cellular Defense against Oxidative Stress in Clonal β-Cells

2001 • 141 citations

Mitochondrial Localization of Mutant Superoxide Dismutase 1 Triggers Caspase-dependent Cell Death in a Cellular Model of Familial Amyotrophic Lateral Sclerosis

2002 • 136 citations

Fatty acid‐induced uncoupling of oxidative phosphorylation is partly due to opening of the mitochondrial permeability transition pore

1998 • 120 citations

Regulation of Cellular Oncosis by Uncoupling Protein 2

2002 • 119 citations

Blood oxidative stress in amyotrophic lateral sclerosis

2000 • 100 citations

Amyotrophic lateral sclerosis is a multifactorial disease

1995 • 99 citations

High Level of Uncoupling Protein 1 Expression in Muscle of Transgenic Mice Selectively Affects Muscles at Rest and Decreases Their IIb Fiber Content

2002 • 60 citations

Changes in myosin mRNA and protein expression in denervated rat soleus and tibialis anterior

1998 • 60 citations

Superoxide dismutase in familial amyotrophic lateral sclerosis: models for gain of function

1995 • 59 citations

Induction of acetylcholine receptor alpha-subunit gene expression in chicken myotubes by blocking electrical activity requires ongoing protein synthesis.

1990 • 23 citations

Partial transformation from fast to slow muscle fibers induced by deafferentation of capsaicin-sensitive muscle afferents

1997 • 8 citations

Denervation Is Not a Primary Cause of Prion Protein Down‐Regulation Occurring in the Spinal Cord of a Transgenic Model of Amyotrophic Lateral Sclerosis

2002 • 4 citations