Amyotrophic Lateral Sclerosis

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

1993 • 6,762 citations

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

1995 • 1,430 citations

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

1996 • 1,261 citations

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

1998 • 1,207 citations

Evidence of Increased Oxidative Damage in Both Sporadic and Familial Amyotrophic Lateral Sclerosis

1997 • 792 citations

Altered Reactivity of Superoxide Dismutase in Familial Amyotrophic Lateral Sclerosis

1996 • 718 citations

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

1998 • 706 citations

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

2000 • 700 citations

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

1999 • 680 citations

Aberrant RNA Processing in a Neurodegenerative Disease: the Cause for Absent EAAT2, a Glutamate Transporter, in Amyotrophic Lateral Sclerosis

1998 • 663 citations

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

1996 • 654 citations

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

2000 • 604 citations

Increased 3‐nitrotyrosine in both sporadic and familial amyotrophic lateral sclerosis

1997 • 593 citations

Epidemiology of mutations in superoxide dismutase in amyotrophic lateal sclerosis

1997 • 517 citations

Bcl-2: Prolonging Life in a Transgenic Mouse Model of Familial Amyotrophic Lateral Sclerosis

1997 • 482 citations

Variants of the heavy neurofilament subunit are associated with the development of amyotrophic lateral sclerosis

1994 • 458 citations

A New Variant of Charcot-Marie-Tooth Disease Type 2 Is Probably the Result of a Mutation in the Neurofilament-Light Gene

2000 • 405 citations

SOD1 mutants linked to amyotrophic lateral sclerosis selectively inactivate a glial glutamate transporter

1999 • 369 citations

Restricted Expression of G86R Cu/Zn Superoxide Dismutase in Astrocytes Results in Astrocytosis But Does Not Cause Motoneuron Degeneration

2000 • 364 citations

A mutant neurofilament subunit causes massive, selective motor neuron death: Implications for the pathogenesis of human motor neuron disease

1994 • 356 citations

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

1997 • 355 citations

Deletions of the heavy neurofilament subunit tail in amyotrophic lateral sclerosis

1999 • 355 citations

Neuronal Death in Amyotrophic Lateral Sclerosis Is Apoptosis

1999 • 354 citations

Caspase-1 and -3 are sequentially activated in motor neuron death in Cu,Zn superoxide dismutase-mediated familial amyotrophic lateral sclerosis

2000 • 346 citations

Expression of a Dominant Negative Mutant of Interleukin-1β Converting Enzyme in Transgenic Mice Prevents Neuronal Cell Death Induced by Trophic Factor Withdrawal and Ischemic Brain Injury

1997 • 345 citations

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

2000 • 326 citations

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

1999 • 324 citations

Linkage of Familial Amyotrophic Lateral Sclerosis With Frontotemporal Dementia to Chromosome 9q21-q22

2000 • 316 citations

Fine Structural Study of Neurofibrillary Changes in a Family with Amyotrophic Lateral Sclerosis

1984 • 300 citations

Elevated free nitrotyrosine levels, but not protein-bound nitrotyrosine or hydroxyl radicals, throughout amyotrophic lateral sclerosis (ALS)-like disease implicate tyrosine nitration as an aberrant in vivo property of one familial ALS-linked superoxide dismutase 1 mutant

1997 • 287 citations

Late Onset Death of Motor Neurons in Mice Overexpressing Wild-Type Peripherin

1999 • 239 citations

SOD1 mutation is assosiated with accumulation of neurofilaments in amyotrophic lateral scelaries

1996 • 237 citations

Absence of neurofilaments reduces the selective vulnerability of motor neurons and slows disease caused by a familial amyotrophic lateral sclerosis-linked superoxide dismutase 1 mutant

1998 • 231 citations

From Charcot to SOD1

1999 • 224 citations

Inducible Nitric Oxide Synthase Up‐Regulation in a Transgenic Mouse Model of Familial Amyotrophic Lateral Sclerosis

1999 • 224 citations

Caspase-1 is activated in neural cells and tissue with amyotrophic lateral sclerosis-associated mutations in copper-zinc superoxide dismutase

1998 • 218 citations

Protective effect of neurofilament heavy gene overexpression in motor neuron disease induced by mutant superoxide dismutase

1998 • 208 citations

Linkage of recessive familial amyotrophic lateral sclerosis to chromosome 2q33–q35

1994 • 202 citations

Linkage of the Gene for an Autosomal Dominant Form of Juvenile Amyotrophic Lateral Sclerosis to Chromosome 9q34

1998 • 188 citations

Characterization of Neuronal Intermediate Filament Protein Expression in Cervical Spinal Motor Neurons in Sporadic Amyotrophic Lateral Sclerosis (ALS)

2000 • 176 citations

Detection and cellular localization of enterovirus RNA sequences in spinal cord of patients with ALS

2000 • 174 citations

Glutamate Slows Axonal Transport of Neurofilaments in Transfected Neurons

2000 • 172 citations

Elevated “Hydroxyl Radical” Generation In Vivo in an Animal Model of Amyotrophic Lateral Sclerosis

1998 • 171 citations

Amyotrophic Lateral Sclerosis-linked Glutamate Transporter Mutant Has Impaired Glutamate Clearance Capacity

2001 • 171 citations

Linkage of a commoner form of recessive amyotrophic lateral sclerosis to chromosome 15q15-q22 markers

1998 • 169 citations

Bax and Bcl‐2 Interaction in a Transgenic Mouse Model of Familial Amyotrophic Lateral Sclerosis

1999 • 162 citations

Peripherin and Neurofilament Protein Coexist in Spinal Spheroids of Motor Neuron Disease

1992 • 156 citations

Astrocytes interact intimately with degenerating motor neurons in mouse amyotrophic lateral sclerosis (ALS)

1999 • 138 citations

Human umbilical cord blood effect on sod mice (amyotrophic lateral sclerosis)

2000 • 138 citations

Increased motoneuron survival and improved neuromuscular function in transgenic ALS mice after intraspinal injection of an adeno-associated virus encoding Bcl-2

2000 • 137 citations

Intracellular Calcium Parallels Motoneuron Degeneration in SOD-1 Mutant Mice

1998 • 136 citations

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

1999 • 128 citations

Mutations in the glutamate transporter EAAT2 gene do not cause abnormal EAAT2 transcripts in amyotrophic lateral sclerosis

1998 • 113 citations

Formation of Intermediate Filament Protein Aggregates with Disparate Effects in Two Transgenic Mouse Models Lacking the Neurofilament Light Subunit

2000 • 101 citations

Pathogenesis of two axonopathies does not require axonal neurofilaments

1998 • 97 citations

神経フィラメントの欠如は,家族性筋萎縮性側索硬化症関連スーパオキジドジスムターゼ1変異に対する運動ニューロンの選択的易損性を低下させ,疾患を遅らせる

1998 • 93 citations

Extra Neurofilament NF-L Subunits Rescue Motor Neuron Disease Caused by Overexpression of the Human NF-H Gene in Mice

1999 • 88 citations

Overexpression of neurofilament subunit NF-L and NF-H extends survival of a mouse model for amyotrophic lateral sclerosis

2000 • 88 citations

Chronic Mitochondrial Inhibition Induces Selective Motoneuron Death In Vitro: A New Model for Amyotrophic Lateral Sclerosis

2000 • 86 citations

Lack of involvement of neuronal nitric oxide synthase in the pathogenesis of a transgenic mouse model of familial amyotrophic lateral sclerosis

1999 • 86 citations

The prostate apoptosis response‐4 protein participates in motor neuron degeneration in amyotrophic lateral sclerosis

2000 • 82 citations

In vitro induction of neuronal apoptosis by anti-Fas antibody-containing sera from amyotrophic lateral sclerosis patients

2000 • 78 citations

Nitration of the Low Molecular Weight Neurofilament Is Equivalent in Sporadic Amyotrophic Lateral Sclerosis and Control Cervical Spinal Cord

1998 • 78 citations

Partial deficiency of manganese superoxide dismutase exacerbates a transgenic mouse model of amyotrophic lateral sclerosis

2000 • 75 citations

Reduction of axonal caliber does not alleviate motor neuron disease caused by mutant superoxide dismutase 1

2000 • 69 citations

Differential Screening of Mutated SOD1 Transgenic Mice Reveals Early Up-Regulation of a Fast Axonal Transport Component in Spinal Cord Motor Neurons

2000 • 57 citations

Toxicity of ALS-Linked SOD1 Mutants

2000 • 51 citations

Absence of p53: No Effect in a Transgenic Mouse Model of Familial Amyotrophic Lateral Sclerosis

2000 • 48 citations

Altered Ionic Conductances in Axons of Transgenic Mouse Expressing the Human Neurofilament Heavy Gene: A Mouse Model of Amyotrophic Lateral Sclerosis

2000 • 40 citations

Response

1999 • 34 citations

Amyotrophic Lateral Sclerosis Immunoglobulins Increase Intracellular Calcium in a Motoneuron Cell Line

1997 • 33 citations

Bcl-2 Overexpression Does Not Protect Neurons from Mutant Neurofilament-Mediated Motor Neuron Degeneration

1999 • 30 citations

DNA laddering and caspase 3-like activity in the spinal cord of a mouse model of familial amyotrophic lateral sclerosis.

2000 • 23 citations

Extra Axonal Neurofilaments Do Not Exacerbate Disease Caused by Mutant Cu,Zn Superoxide Dismutase

2000 • 21 citations