Current hypotheses for the underlying biology of 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

TDP-43 Mutations in Familial and Sporadic Amyotrophic Lateral Sclerosis

2008 • 2,514 citations

Knockout of Glutamate Transporters Reveals a Major Role for Astroglial Transport in Excitotoxicity and Clearance of Glutamate

1996 • 2,470 citations

TARDBP mutations in individuals with sporadic and familial amyotrophic lateral sclerosis

2008 • 1,468 citations

β-Lactam antibiotics offer neuroprotection by increasing glutamate transporter expression

2005 • 1,433 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

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

2001 • 1,373 citations

ALS: A Disease of Motor Neurons and Their Nonneuronal Neighbors

2006 • 1,371 citations

UNRAVELING THE MECHANISMS INVOLVED IN MOTOR NEURON DEGENERATION IN ALS

2004 • 1,362 citations

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

1997 • 1,313 citations

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

1998 • 1,207 citations

Deletion of the hypoxia-response element in the vascular endothelial growth factor promoter causes motor neuron degeneration

2001 • 1,012 citations

Mutant dynactin in motor neuron disease

2003 • 933 citations

Retrograde Viral Delivery of IGF-1 Prolongs Survival in a Mouse ALS Model

2003 • 863 citations

VEGF is a modifier of amyotrophic lateral sclerosis in mice and humans and protects motoneurons against ischemic death

2003 • 858 citations

Focal loss of the glutamate transporter EAAT2 in a transgenic rat model of SOD1 mutant-mediated amyotrophic lateral sclerosis (ALS)

2002 • 822 citations

Altered Reactivity of Superoxide Dismutase in Familial Amyotrophic Lateral Sclerosis

1996 • 718 citations

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

2000 • 700 citations

TARDBP mutations in amyotrophic lateral sclerosis with TDP-43 neuropathology: a genetic and histopathological analysis

2008 • 686 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

Abnormal excitatory amino acid metabolism in amyotrophic lateral sclerosis

1990 • 630 citations

Slowing of axonal transport is a very early event in the toxicity of ALS–linked SOD1 mutants to motor neurons

1999 • 621 citations

TDP‐43 A315T mutation in familial motor neuron disease

2008 • 596 citations

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

1997 • 593 citations

VEGF delivery with retrogradely transported lentivector prolongs survival in a mouse ALS model

2004 • 591 citations

Disturbance of Nuclear and Cytoplasmic TAR DNA-binding Protein (TDP-43) Induces Disease-like Redistribution, Sequestration, and Aggregate Formation

2008 • 589 citations

Increased expression of neurofilament subunit NF-L produces morphological alterations that resemble the pathology of human motor neuron disease

1993 • 550 citations

Treatment of motoneuron degeneration by intracerebroventricular delivery of VEGF in a rat model of ALS

2004 • 494 citations

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

1997 • 482 citations

Neuron-Specific Expression of Mutant Superoxide Dismutase 1 in Transgenic Mice Does Not Lead to Motor Impairment

2001 • 469 citations

Multiple roles of TDP-43 in gene expression, splicing regulation, and human disease

2007 • 468 citations

TDP-43 Binds Heterogeneous Nuclear Ribonucleoprotein A/B through Its C-terminal Tail

2005 • 440 citations

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

2006 • 382 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

Effect of recombinant human insulin-like growth factor-I on progression of ALS

1997 • 359 citations

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

1994 • 356 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

A placebo-controlled trial of insulin-like growth factor-I in amyotrophic lateral sclerosis

1998 • 338 citations

Deregulation of Cdk5 in a Mouse Model of ALS

2001 • 336 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

Protein Oxidative Damage in a Transgenic Mouse Model of Familial Amyotrophic Lateral Sclerosis

1998 • 287 citations

Mutant SOD1 causes motor neuron disease independent of copper chaperone–mediated copper loading

2002 • 275 citations

Increased expression of the glial glutamate transporter EAAT2 modulates excitotoxicity and delays the onset but not the outcome of ALS in mice

2003 • 269 citations

Glutamate levels in cerebrospinal fluid in amyotrophic lateral sclerosis: a reappraisal using a new HPLC method with coulometric detection in a large cohort of patients

2002 • 268 citations

Up‐Regulation of Protein Chaperones Preserves Viability of Cells Expressing Toxic Cu/Zn‐Superoxide Dismutase Mutants Associated with Amyotrophic Lateral Sclerosis

1999 • 262 citations

Neuroprotective Effects of Glial Cell Line-Derived Neurotrophic Factor Mediated by an Adeno-Associated Virus Vector in a Transgenic Animal Model of Amyotrophic Lateral Sclerosis

2002 • 254 citations

Depletion of TDP 43 overrides the need for exonic and intronic splicing enhancers in the human apoA-II gene

2005 • 218 citations

Benefit of valproic acid in suppressing disease progression of ALS model mice

2004 • 213 citations

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

1998 • 208 citations

Dorfin Ubiquitylates Mutant SOD1 and Prevents Mutant SOD1-mediated Neurotoxicity

2002 • 192 citations

Common molecular signature in SOD1 for both sporadic and familial amyotrophic lateral sclerosis

2007 • 189 citations

Vascular Endothelial Growth Factor Overexpression Delays Neurodegeneration and Prolongs Survival in Amyotrophic Lateral Sclerosis Mice

2007 • 162 citations