l-Carnitine suppresses the onset of neuromuscular degeneration and increases the life span of mice with familial 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

Carnitine--metabolism and functions

1983 • 1,620 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

From charcot to lou gehrig: deciphering selective motor neuron death in als

2001 • 1,373 citations

Mitochondrial permeability transition: a common pathway to necrosis and apoptosis

2003 • 748 citations

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

1998 • 706 citations

Molecular and Functional Identification of Sodium Ion-dependent, High Affinity Human Carnitine Transporter OCTN2

1998 • 694 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

Mitochondrial disease in mouse results in increased oxidative stress

1999 • 634 citations

CARNITINE

1988 • 610 citations

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

2002 • 596 citations

Neuroprotective role of adenosine in cerebral ischaemia

1992 • 450 citations

Amyotrophic Lateral Sclerosis

2001 • 437 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

Phospholipase A₂ and Its Role in Brain Tissue

1997 • 337 citations

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

1999 • 324 citations

Feeding acetyl- <scp>l</scp> -carnitine and lipoic acid to old rats significantly improves metabolic function while decreasing oxidative stress

2002 • 315 citations

Acetyl-L-carnitine physical-chemical, metabolic, and therapeutic properties: relevance for its mode of action in Alzheimer's disease and geriatric depression

2000 • 304 citations

Cyclosporin and carnitine prevent the anoxic death of cultured hepatocytes by inhibiting the mitochondrial permeability transition

1993 • 294 citations

Nutritional status of patients with amyotrophic lateral sclerosis: relation to the proximity of death

1996 • 290 citations

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

2000 • 252 citations

Impairment of mitochondrial function in skeletal muscle of patients with amyotrophic lateral sclerosis

1998 • 249 citations

Long‐term acetyl‐L‐carnitine treatment in Alzheimer's disease

1991 • 233 citations

Delaying Brain Mitochondrial Decay and Aging with Mitochondrial Antioxidants and Metabolites

2002 • 206 citations

Mitochondrial involvement in amyotrophic lateral sclerosis

2002 • 203 citations

Insulin-like growth factor-1 is a potent neuronal rescue agent after hypoxic-ischemic injury in fetal lambs.

1996 • 182 citations

Mitochondrial permeability transition in acute neurodegeneration

2002 • 180 citations

l-Carnitine inhibits cisplatin-induced injury of the kidney and small intestine

2002 • 173 citations

Antiradical effects in l-propionyl carnitine protection of the heart against ischemia-reperfusion injury: The possible role of iron chelation

1992 • 169 citations

Molecular analysis of the muscle pathology associated with mitochondrial DNA deletions

1992 • 166 citations

Functional relevance of carnitine transporter OCTN2 to brain distribution of<scp>l</scp>‐carnitine and acetyl‐<scp>l</scp>‐carnitine across the blood–brain barrier

2001 • 155 citations

Amyotrophic lateral sclerosis

1996 • 140 citations

Hepatic Ultrastructural Changes and Liver Dysfunction in Amyotrophic Lateral Sclerosis

1987 • 133 citations

Roles of long chain fatty acids and carnitine in mitochondrial membrane permeability transition11Abbreviations: CsA, cyclosporin A; Cyt. c, cytochrome c; FBS, fetal bovine serum; Pal-CoA, palmitoyl-CoA; Pi, inorganic phosphate; LCFA, long chain fatty acid; MPT, membrane permeability transition; PLA2, phospholipase A2; RCR, respiratory control ratio; and T3, 3,3′,5-triiodothyronine.

2001 • 130 citations

Oxidative Stress Underlies the Mechanism for Ca²⁺-induced Permeability Transition of Mitochondria

2003 • 128 citations

Influence of Severe Hypoglycemia on Brain Extracellular Calcium and Potassium Activities, Energy, and Phospholipid Metabolism

1984 • 126 citations

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

2003 • 121 citations

Hypoglycemia, brain metabolism, and brain damage

1988 • 115 citations

Cerebral Extracellular Calcium Activity in Severe Hypoglycemia: Relation to Extracellular Potassium and Energy State

1984 • 112 citations

Animal model of dementia induced by entorhinal synaptic damage and partial restoration of cognitive deficits by BDNF and carnitine

2002 • 105 citations

Free Fatty Acid Effects on Mitochondrial Permeability: An Overview

2001 • 92 citations

The carnitine acyltransferases: modulators of acyl-CoA-dependent reactions

2000 • 87 citations

Anti-apoptotic Effect of Acetyl-l-carnitine and l-Carnitine in Primary Cultured Neurons.

2000 • 86 citations

L‐carnitine inhibits hepatocarcinogenesis via protection of mitochondria

2004 • 84 citations

Neuronal protection from glucose deprivation via modulation of glucose transport and inhibition of apoptosis: a role for the insulin-like growth factor system

2004 • 82 citations

Pharmacokinetics of IV and oral acetyl-L-carnitine in a multiple dose regimen in patients with senile dementia of Alzheimer type

1992 • 79 citations

Neuroprotective activity of acetyl‐L‐carnitine: Studies in vitro

1994 • 74 citations

Protective effect ofL-carnitine in ammonia-precipitated encephalopathy in the portacaval shunted rat

1997 • 65 citations

L-carnitine attenuates doxorubicin-induced lipid peroxidation in rats

1999 • 61 citations

Cross Talk of Nitric Oxide, Oxygen Radicals, and Superoxide Dismutase Regulates the Energy Metabolism and Cell Death and Determines the Fates of Aerobic Life

2003 • 54 citations

Acetyl-l-carnitine shows neuroprotective and neurotrophic activity in primary culture of rat embryo motoneurons

2002 • 52 citations

l-Carnitine inhibits hypoglycemia-induced brain damage in the rat

2005 • 46 citations

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

2001 • 43 citations

Prevention of ammonia toxicity byl-carnitine: metabolic changes in brain

1984 • 43 citations

Characteristics of Cardiac Hypertrophy in the Juvenile Visceral Steatosis Mouse with Systemic Carnitine Deficiency

1998 • 35 citations

L-Carnitine Changes the Levels of Insulin-like Growth Factors (IGFs) and IGF Binding Proteins in Streptozotocin-induced Diabetic Rat.

2001 • 35 citations

Carnitines Increase Plasma Levels of Adenosine and ATP in Humans

1997 • 23 citations

Cardiomegaly in the juvenile visceral steatosis (JVS) mouse is reduced with acute elevation of heart short‐chain acyl‐carnitine level after <scp>l</scp>‐carnitine injection

1999 • 16 citations

Anti-apoptotic Effect of Acetyl-/-carnitine and l-Carnitine in Primary Cultured Neurons

2000 • 13 citations