Excitotoxicity, Genetics and Neurodegeneration in Amyotrophic Lateral Sclerosis

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

1993 • 6,762 citations

GLUTATHIONE

1983 • 5,487 citations

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

1994 • 3,959 citations

Oxidative Stress, Glutamate, and Neurodegenerative Disorders

1993 • 3,746 citations

A Controlled Trial of Riluzole in Amyotrophic Lateral Sclerosis

1994 • 2,067 citations

Normal oxidative damage to mitochondrial and nuclear DNA is extensive.

1988 • 1,709 citations

Selective loss of glial glutamate transporter GLT‐1 in amyotrophic lateral sclerosis

1995 • 1,430 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

ALS-Linked SOD1 Mutant G85R Mediates Damage to Astrocytes and Promotes Rapidly Progressive Disease with SOD1-Containing Inclusions

1997 • 1,313 citations

Motor neurons in Cu/Zn superoxide dismutase-deficient mice develop normally but exhibit enhanced cell death after axonal injury

1996 • 1,261 citations

Dose-ranging study of riluzole in amyotrophic lateral sclerosis

1996 • 1,248 citations

MITOCHONDRIAL DNA MUTATIONS AS AN IMPORTANT CONTRIBUTOR TO AGEING AND DEGENERATIVE DISEASES

1989 • 1,179 citations

Decreased Glutamate Transport by the Brain and Spinal Cord in Amyotrophic Lateral Sclerosis

1992 • 1,170 citations

An excitatory amino-acid transporter with properties of a ligand-gated chloride channel

1995 • 1,124 citations

Divalent ion permeability of AMPA receptor channels is dominated by the edited form of a single subunit

1992 • 1,077 citations

Functional comparisons of three glutamate transporter subtypes cloned from human motor cortex

1994 • 988 citations

Glutamate toxicity in a neuronal cell line involves inhibition of cystine transport leading to oxidative stress

1989 • 987 citations

Altered Reactivity of Superoxide Dismutase in Familial Amyotrophic Lateral Sclerosis

1996 • 718 citations

The oxidative inactivation of mitochondrial electron transport chain components and ATPase.

1990 • 718 citations

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

1995 • 694 citations

Benefit of vitamin E, riluzole, and gababapentin in a transgenic model of familial amyotrophic lateral sclerosis

1996 • 654 citations

Abnormal excitatory amino acid metabolism in amyotrophic lateral sclerosis

1990 • 630 citations

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

1993 • 627 citations

Chronic inhibition of glutamate uptake produces a model of slow neurotoxicity.

1993 • 623 citations

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

1993 • 583 citations

AMPA and kainate receptors

1995 • 460 citations

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)

1995 • 435 citations

Transgenic mice carrying a human mutant superoxide dismutase transgene develop neuronal cytoskeletal pathology resembling human amyotrophic lateral sclerosis lesions.

1996 • 397 citations

The role of calcium‐binding proteins in selective motoneuron vulnerability in amyotrophic lateral sclerosis

1994 • 360 citations

Aggregation of Mutant Cu/Zn Superoxide Dismutase Proteins in a Culture Model of ALS

1997 • 355 citations

Amyotrophic lateral sclerosis: Recent insights from genetics and transgenic mice

1995 • 353 citations

Oxidative damage to mitochondrial DNA and its relationship to ageing

1995 • 350 citations

Mutations associated with amyotrophic lateral sclerosis convert superoxide dismutase from an antiapoptotic gene to a proapoptotic gene: studies in yeast and neural cells.

1995 • 334 citations

Oxidative damage to protein in sporadic motor neuron disease spinal cord

1995 • 326 citations

Age-Dependent Penetrance of Disease in a Transgenic Mouse Model of Familial Amyotrophic Lateral Sclerosis

1995 • 317 citations

Superoxide dismutase is an abundant component in cell bodies, dendrites, and axons of motor neurons and in a subset of other neurons.

1995 • 313 citations

Glutamate, excitotoxicity and amyotrophic lateral sclerosis

1997 • 294 citations

Mitochondria and Na+/Ca2+ exchange buffer glutamate-induced calcium loads in cultured cortical neurons

1995 • 292 citations

Chronic inhibition of superoxide dismutase produces apoptotic death of spinal neurons.

1994 • 291 citations

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

1993 • 279 citations

Ubiquitin deposits in anterior horn cells in motor neurone disease

1988 • 277 citations

Down-regulation of copper/zinc superoxide dismutase causes apoptotic death in PC12 neuronal cells.

1994 • 271 citations

Superoxide dismutase protects calcineurin from inactivation

1996 • 270 citations

CSF and Plasma Amino Acid Levels in Motor Neuron Disease: Elevation of CSF Glutamate in a Subset of Patients

1995 • 264 citations

Cortical function in amyotrophic lateral sclerosis

1993 • 254 citations

Cytoarchitectonic organization of the spinal cord in the rat: II. The cervical and upper thoracic cord

1989 • 253 citations

Glutamate transporter gene expression in amyotrophic lateral sclerosis motor cortex

1996 • 237 citations

Contributory mechanisms in the causation of neurodegenerative disorders

1993 • 228 citations

A filamentous inclusion body within anterior horn neurones in motor neurone disease defined by immunocytochemical localisation of ubiquitin

1988 • 226 citations

Calcium‐permeable α‐amino‐3‐hydroxy‐5‐methyl‐4‐isoxazole propionic acid receptors: A molecular determinant of selective vulnerability in amyotrophic lateral sclerosis

1997 • 210 citations

The neuroexcitotoxic amino acids glutamate and aspartate are altered in the spinal cord and brain in amyotrophic lateral sclerosis

1988 • 210 citations

Free Radicals in Diagnostic Medicine

1994 • 204 citations

Cell culture evidence for neuronal degeneration in amyotrophic lateral sclerosis being linked to glutamate AMPA/kainate receptors

1993 • 189 citations

Glutamine from Glial Cells Is Essential for the Maintenance of the Nerve Terminal Pool of Glutamate: Immunogold Evidence from Hippocampal Slice Cultures

1995 • 189 citations

Excitotoxic mechanisms in the pathogenesis of amyotrophic lateral sclerosis.

1995 • 174 citations

Glutamate uptake is inhibited by arachidonic acid and oxygen radicals via two distinct and additive mechanisms.

1994 • 174 citations

Cortical Excitability in Amyotrophic Lateral Sclerosis: A Clue to Pathogenesis

1993 • 173 citations

Glutamate and Glutamine Metabolism and Compartmentation in Astrocytes

1993 • 173 citations

Amyotrophic lateral sclerosis: Amino acid levels in plasma and cerebrospinal fluid

1990 • 163 citations

Placebo-controlled trial of gabapentin in patients with amyotrophic lateral sclerosis

1996 • 153 citations

Superoxide Dismutase 1 Subunits with Mutations Linked to Familial Amyotrophic Lateral Sclerosis Do Not Affect Wild-type Subunit Function

1995 • 149 citations

Familial amyotrophic lateral sclerosis with a mutation in exon 4 of the Cu/Zn superoxide dismutase gene: pathological and immunocytochemical changes

1996 • 130 citations

Brain glutamate deficiency in amyotrophic lateral sclerosis

1987 • 118 citations

Expression of the glial glutamate transporter EAAT2 in the human CNS: an immunohistochemical study

1997 • 116 citations

AMPA receptor desensitization predicts the selective vulnerability of cerebellar Purkinje cells to excitotoxicity

1995 • 111 citations

Modulation of embryonic chick motoneuron glutamate sensitivity by interneurons and agonists

1986 • 105 citations

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

1991 • 98 citations

Iron, selenium and glutathione peroxidase activity are elevated in sporadic motor neuron disease

1994 • 98 citations

Molecular genetic basis of familial ALS

1996 • 97 citations

Excitotoxicity and motor neurone disease: A review of the evidence

1994 • 96 citations

Motor neuron disease

1995 • 96 citations

Glutamate receptor update

1994 • 95 citations

Anti-Glutamate Therapy in Amyotrophic Lateral Sclerosis: A Trial Using Lamotrigine

1993 • 92 citations

Inactivation of mammalian brain glutamine synthetase by oxygen radicals

1988 • 75 citations

The quantitative autoradiographic distribution of [3H]MK-801 binding sites in the normal human spinal cord

1991 • 74 citations

Molecular cloning of human brain glutamate/aspartate transporter II

1994 • 74 citations

Glutamate or aspartate as a possible neurotransmitter of cerebral corticofugal fibers in the monkey

1983 • 70 citations

Cloning and characterization of a glutamate transporter cDNA from human cerebellum

1993 • 64 citations

[3H]d-aspartate binding sites in the normal human spinal cord and changes in motor neuron disease: a quantitative autoradiographic study

1994 • 62 citations

Neuron-specific human glutamate transporter: molecular cloning, characterization and expression in human brain

1994 • 59 citations

Metallothionein in Amyotrophic Lateral Sclerosis

1994 • 56 citations

Assessment of reliability and biological significance of glutamate levels in cerebrospinal fluid

1993 • 54 citations

Neuropathology of sporadic amyotrophic lateral sclerosis of long duration

1997 • 54 citations

Nonverbal visual attention, but not recognition memory or learning, processes are impaired in motor neurone disease

1996 • 52 citations

Abnormal distribution of phosphorylated neurofilaments in neuronal degeneration induced by kainic acid

1990 • 48 citations

Non-NMDA receptors in motor neuron disease (MND): a quantitative autoradiographic study in spinal cord and motor cortex using [3H]CNQX and [3H]kainate

1994 • 44 citations

Immunocytochemical study of the distribution of the free radical scavenging enzymes CU/ZN superoxide dismutase (SOD1); MN superoxide dismutase (MN SOD) and catalase in the normal human spinal cord and in motor neuron disease

1997 • 43 citations

AN IMMUNOCYTOCHEMICAL STUDY OF THE DISTRIBUTION OF AMPA SELECTIVE GLUTAMATE RECEPTOR SUBUNITS IN THE NORMAL HUMAN MOTOR SYSTEM

1996 • 43 citations

Copper/zinc superoxide dismutase mRNA levels are increased in sporadic amyotrophic lateral sclerosis motorneurons

1994 • 42 citations

Distribution of AMPA-selective glutamate receptor subunits in the human hippocampus and cerebellum

1995 • 41 citations

Parkinsonism in motor neuron disease: case report and literature review

1995 • 40 citations

Excitatory amino acids, free radicals and the pathogenesis of motor neuron disease

1994 • 38 citations

N-methyl-d-aspartate (NMDA) receptors in the spinal cord and motor cortex in motor neuron disease: a quantitative autoradiographic study using [3H]MK-801

1994 • 33 citations

Multimodality Evoked Potentials in Motor Neuron Disease

1990 • 28 citations

Autoradiographic distribution of binding sites for the non-NMDA receptor antagonist [3H]CNQX in human motor cortex, brainstem and spinal cord

1993 • 27 citations

A quantitative autoradiographic study of [3H]kainate binding sites in the normal human spinal cord, brainstem and motor cortex

1994 • 19 citations

Selective loss of neurofilament proteins after exposure of differentiated human IMR-32 neuroblastoma cells to oxidative stress

1996 • 14 citations

Free Radicals and the Pathogenesis of Neuronal Death: Cooperative Role of Excitatory Amino Acids

1994 • 10 citations

NMDA-induced whole-cell currents and single channel conductances in tectal neurons during two stages of early development of chicken

1995 • 8 citations

Diseases of the Nervous System: Clinical Neurobiology. Vol.2

1986 • 5 citations

Diseases of the Nervous System: Clinical Neurobiology. Vol.1

1986 • 2 citations

Deleted Work

1955 • 0 citations