Amyotrophic lateral sclerosis models and human neuropathology: similarities and differences

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

1993 • 6,762 citations

Ubiquitinated TDP-43 in Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis

2006 • 5,928 citations

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

1994 • 3,959 citations

TDP-43 is a component of ubiquitin-positive tau-negative inclusions in frontotemporal lobar degeneration and amyotrophic lateral sclerosis

2006 • 2,449 citations

Onset and Progression in Inherited ALS Determined by Motor Neurons and Microglia

2006 • 1,540 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

Guanine nucleotide exchange factors for Rho GTPases: turning on the switch

2002 • 1,225 citations

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

1998 • 1,207 citations

Astrocytes expressing ALS-linked mutated SOD1 release factors selectively toxic to motor neurons

2007 • 1,160 citations

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

2002 • 822 citations

The gene encoding alsin, a protein with three guanine-nucleotide exchange factor domains, is mutated in a form of recessive amyotrophic lateral sclerosis

2001 • 722 citations

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

1998 • 706 citations

Wild-type microglia extend survival in PU.1 knockout mice with familial amyotrophic lateral sclerosis

2006 • 698 citations

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

1995 • 694 citations

A gene encoding a putative GTPase regulator is mutated in familial amyotrophic lateral sclerosis 2

2001 • 660 citations

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

1993 • 550 citations

Progressive neuronopathy in transgenic mice expressing the human neurofilament heavy gene: A mouse model of amyotrophic lateral sclerosis

1993 • 549 citations

Disruption of Dynein/Dynactin Inhibits Axonal Transport in Motor Neurons Causing Late-Onset Progressive Degeneration

2002 • 530 citations

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

2001 • 469 citations

Defective axonal transport in a transgenic mouse model of amyotrophic lateral sclerosis

1995 • 464 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

Conversion to the amyotrophic lateral sclerosis phenotype is associated with intermolecular linked insoluble aggregates of SOD1 in mitochondria

2006 • 419 citations

Accumulation of SOD1 Mutants in Postnatal Motoneurons Does Not Cause Motoneuron Pathology or Motoneuron Disease

2002 • 409 citations

Point mutations of the p150 subunit of dynactin ( DCTN1 ) gene in ALS

2004 • 409 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

Rats Expressing Human Cytosolic Copper–Zinc Superoxide Dismutase Transgenes with Amyotrophic Lateral Sclerosis: Associated Mutations Develop Motor Neuron Disease

2001 • 316 citations

Neurodegeneration : the molecular pathology of dementia and movement disorders

2003 • 294 citations

Intense Superoxide Dismutase-1 Immunoreactivity in Intracytoplasmic Hyaline Inclusions of Familial Amyotrophic Lateral Sclerosis with Posterior Column Involvement

1996 • 289 citations

TDP-43 immunoreactivity in neuronal inclusions in familial amyotrophic lateral sclerosis with or without SOD1 gene mutation

2007 • 288 citations

Minute quantities of misfolded mutant superoxide dismutase‐1 cause amyotrophic lateral sclerosis

2003 • 250 citations

ALS-associated mutant SOD1G93A causes mitochondrial vacuolation by expansion of the intermembrane space and by involvement of SOD1 aggregation and peroxisomes

2003 • 247 citations

ALS2, a novel guanine nucleotide exchange factor for the small GTPase Rab5, is implicated in endosomal dynamics

2003 • 245 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

Familial Amyotrophic Lateral Sclerosis

1967 • 235 citations

Fibrillar Inclusions and Motor Neuron Degeneration in Transgenic Mice Expressing Superoxide Dismutase 1 with a Disrupted Copper-Binding Site

2002 • 233 citations

Familial amyotrophic lateral sclerosis

2002 • 231 citations

The Golgi apparatus of spinal cord motor neurons in transgenic mice expressing mutant Cu,Zn superoxide dismutase becomes fragmented in early, preclinical stages of the disease.

1996 • 224 citations

Infantile-Onset Ascending Hereditary Spastic Paralysis Is Associated with Mutations in the Alsin Gene

2002 • 211 citations

Pathology of amyotrophic lateral sclerosis.

1982 • 209 citations

Isolation and characterization of the active cDNA of the human cell cycle gene (RCC1) involved in the regulation of onset of chromosome condensation.

1987 • 208 citations

Neurodegeneration: The Molecular Pathology of Dementia and Movement Disorders

2004 • 203 citations

Lessons from models of SOD1-linked familial ALS

2004 • 193 citations

A low expressor line of transgenic mice carrying a mutant human Cu,Zn superoxide dismutase ( SOD1 ) gene develops pathological changes that most closely resemble those in human amyotrophic lateral sclerosis

1997 • 189 citations

Absence of the Mid-sized Neurofilament Subunit Decreases Axonal Calibers, Levels of Light Neurofilament (NF-L), and Neurofilament Content

1998 • 181 citations

New consensus research on neuropathological aspects of familial amyotrophic lateral sclerosis with superoxide dismutase 1 (SOD1) gene mutations: Inclusions containing SOD1 in neurons and astrocytes

2000 • 172 citations

HEREDITARY MOTOR SYSTEM DISEASES (CHRONIC JUVENILE AMYOTROPHIC LATERAL SCLEROSIS)

1990 • 167 citations

Gene transfer demonstrates that muscle is not a primary target for non-cell-autonomous toxicity in familial amyotrophic lateral sclerosis

2006 • 154 citations

Disruption of Type IV Intermediate Filament Network in Mice Lacking the Neurofilament Medium and Heavy Subunits

1999 • 142 citations

Amyotrophic Lateral Sclerosis Associated with Genetic Abnormalities in the Gene Encoding Cu/Zn Superoxide Dismutase: Molecular Pathology of Five New Cases, and Comparison with Previous Reports and 73 Sporadic Cases of ALS

1998 • 142 citations

SLOW, LATENT AND TEMPERATE VIRUS INFECTIONS

1967 • 141 citations

TDP-43 in differential diagnosis of motor neuron disorders

2007 • 137 citations

Als2 -deficient mice exhibit disturbances in endosome trafficking associated with motor behavioral abnormalities

2006 • 136 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

Mice deficient in the Rab5 guanine nucleotide exchange factor ALS2/alsin exhibit age-dependent neurological deficits and altered endosome trafficking

2005 • 129 citations

Limited corticospinal tract involvement in amyotrophic lateral sclerosis subjects with the A4V mutation in the copper/zinc superoxide dismutase gene

1998 • 127 citations

Lack of TDP-43 abnormalities in mutant SOD1 transgenic mice shows disparity with ALS

2007 • 119 citations

Familial Amyotrophic Lateral Sclerosis with a Two Base Pair Deletion in Superoxide Dismutase 1 Gene: Multisystem Degeneration with Intracytoplasmic Hyaline Inclusions in Astrocytes

1996 • 116 citations

Loss ofALS2Function Is Insufficient to Trigger Motor Neuron Degeneration in Knock-Out Mice But Predisposes Neurons to Oxidative Stress

2005 • 115 citations

Pathological characterization of astrocytic hyaline inclusions in familial amyotrophic lateral sclerosis.

1997 • 113 citations

Differential vulnerability of oculomotor, facial, and hypoglossal nuclei in G86R superoxide dismutase transgenic mice

2000 • 112 citations

Motor Neuron Disease in Mice Expressing the Wild Type-Like D90A Mutant Superoxide Dismutase-1

2006 • 107 citations

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

2000 • 101 citations

Familial amyotrophic lateral sclerosis

1997 • 96 citations

The first nonsense mutation in alsin results in a homogeneous phenotype of infantile‐onset ascending spastic paralysis with bulbar involvement in two siblings

2003 • 94 citations

[On intracellular inclusions in familial amyotrophic lateral sclerosis].

1962 • 90 citations

Quantitative immunocytochemical analysis of the spinal cord in G86R superoxide dismutase transgenic mice: Neurochemical correlates of selective vulnerability

1996 • 90 citations

Familial amyotrophic lateral sclerosis with a point mutation of SOD-1: intrafamilial heterogeneity of disease duration associated with neurofibrillary tangles.

1995 • 86 citations

Degeneration of corticospinal and bulbospinal systems in the superoxide dismutase 1G93A G1H transgenic mouse model of familial amyotrophic lateral sclerosis

2002 • 84 citations

Progressive spinal axonal degeneration and slowness in ALS2‐deficient mice

2006 • 75 citations

Advanced glycation endproduct-modified superoxide dismutase-1 (SOD1)-positive inclusions are common to familial amyotrophic lateral sclerosis patients with SOD1 gene mutations and transgenic mice expressing human SOD1 with a G85R mutation

2000 • 73 citations

Rapid disease progression correlates with instability of mutant SOD1 in familial ALS

2005 • 71 citations

Aggregation of ubiquitin and a mutant ALS-linked SOD1 protein correlate with disease progression and fragmentation of the Golgi apparatus

2000 • 71 citations

Expression of peripherin in ubiquinated inclusions of amyotrophic lateral sclerosis

2003 • 64 citations

Novel missense mutation in ALS2 gene results in infantile ascending hereditary spastic paralysis

2006 • 64 citations

Familial amyotrophic lateral sclerosis with a mutation in the Cu/Zn superoxide dismutase gene

1994 • 63 citations

Redox system expression in the motor neurons in amyotrophic lateral sclerosis (ALS): immunohistochemical studies on sporadic ALS, superoxide dismutase 1 (SOD1)-mutated familial ALS, and SOD1-mutated ALS animal models

2005 • 53 citations

Novel mutation in the ALS2 gene in juvenile amyotrophic lateral sclerosis

2005 • 50 citations

Pathways to motor neuron degeneration in transgenic mouse models

2002 • 50 citations

Mouse motor neuron disease caused by truncated SOD1 with or without C-terminal modification

2005 • 47 citations

Accumulation of Neurofilaments and SOD1-Immunoreactive Products in a Patient With Familial Amyotrophic Lateral Sclerosis With I113T SOD1 Mutation

1999 • 45 citations

Familial Childhood Primary Lateral Sclerosis with Associated Gaze Paresis

1995 • 40 citations

Progressive muscular atrophy variant of familial amyotrophic lateral sclerosis (PMA/ALS)

2000 • 40 citations

Familial Amyotrophic Lateral Sclerosis With a Novel Leu126Ser Mutation in the Copper/Zinc Superoxide Dismutase Gene Showing Mild Clinical Features and Lewy Body–Like Hyaline Inclusions

2001 • 37 citations

Familial amyotrophic lateral sclerosis with His46Arg mutation in Cu/Zn superoxide dismutase presenting characteristic clinical features and Lewy body-like hyaline inclusions

2004 • 37 citations

An In Vitro Model for Lewy Body-Like Hyaline Inclusion/Astrocytic Hyaline Inclusion: Induction by ER Stress with an ALS-Linked SOD1 Mutation

2007 • 36 citations

Recent advances in research on neuropathological aspects of familial amyotrophic lateral sclerosis with superoxide dismutase 1 gene mutations: neuronal Lewy body-like hyaline inclusions and astrocytic hyaline inclusions.

1999 • 35 citations

Familial amyotrophic lateral sclerosis with bulbar onset and a novel Asp101Tyr Cu/Zn superoxide dismutase gene mutation

2004 • 32 citations

A clinicopathological study of a patient with familial amyotrophic lateral sclerosis associated with a two base pair deletion in the copper/zinc superoxide dismutase (SOD1) gene

1997 • 28 citations

Development of a rat model of amyotrophic lateral sclerosis expressing a human SOD1 transgene

2005 • 28 citations

Animal models of amyotrophic lateral sclerosis

2000 • 27 citations

Inverse correlation between the formation of mitochondria-derived vacuoles and Lewy-body-like hyaline inclusions in G93A superoxide-dismutase-transgenic mice

2006 • 16 citations

Familial amyotrophic lateral sclerosis with a point mutation (G37R) of the superoxide dismutase 1 gene: a clinicopathological study

2002 • 14 citations

Numerous conglomerate inclusions in slowly progressive familial amyotrophic lateral sclerosis with posterior column involvement

1999 • 8 citations

Molecular Pathological Studies of Familial Amyotrophic Lateral Sclerosis

1997 • 7 citations

[Clinical features and neuropathological findings of familial amyotrophic lateral sclerosis with an H43R mutation in Cu/Zn superoxide dismutase].

2003 • 6 citations

Histological recovery of the hepatocytes is based on the redox system upregulation in the animal models of mutant superoxide dismutase (SOD)1-linked amyotrophic lateral sclerosis.

2006 • 4 citations

[Amyotrophic lateral sclerosis: recent insights from transgenic animal models with SOD1 mutations].

2004 • 1 citations