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

Aggresomes: A Cellular Response to Misfolded Proteins

1998 • 2,116 citations

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

1997 • 1,313 citations

Degradation of CFTR by the ubiquitin-proteasome pathway

1995 • 1,285 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

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

1995 • 694 citations

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

1999 • 621 citations

Superoxide dismutase 1 with mutations linked to familial amyotrophic lateral sclerosis possesses significant activity.

1994 • 604 citations

Characterization and Dynamics of Aggresome Formation by a Cytosolic Gfp-Chimera✪

1999 • 592 citations

Frameshift Mutants of β Amyloid Precursor Protein and Ubiquitin-B in Alzheimer's and Down Patients

1998 • 558 citations

Intragenic deletion in the gene encoding ubiquitin carboxy-terminal hydrolase in gad mice

1999 • 505 citations

Decreased Zinc Affinity of Amyotrophic Lateral Sclerosis‐Associated Superoxide Dismutase Mutants Leads to Enhanced Catalysis of Tyrosine Nitration by Peroxynitrite

1997 • 483 citations

Intracellular turnover of cystic fibrosis transmembrane conductance regulator. Inefficient processing and rapid degradation of wild-type and mutant proteins.

1994 • 482 citations

A gain-of-function of an amyotrophic lateral sclerosis-associated Cu,Zn-superoxide dismutase mutant: An enhancement of free radical formation due to a decrease in Km for hydrogen peroxide.

1996 • 464 citations

Development of central nervous system pathology in a murine transgenic model of human amyotrophic lateral sclerosis.

1994 • 397 citations

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

1996 • 397 citations

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

1997 • 355 citations

Human motor neuron diseases.

1982 • 352 citations

Specific aggregation of partially folded polypeptide chains: The molecular basis of inclusion body composition

1996 • 307 citations

Neurofilaments and Orthograde Transport Are Reduced in Ventral Root Axons of Transgenic Mice that Express Human SOD1 with a G93A Mutation

1997 • 293 citations

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

1996 • 289 citations

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

1998 • 287 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

Serial analysis of microtubules in cultured rat sensory axons

1981 • 202 citations

Cytopathology of amyotrophic lateral sclerosis.

1991 • 192 citations

Cytoskeletal proteins and Golgi dynamics

1998 • 180 citations

Structural Neurology: Are Seeds at the Root of Neuronal Degeneration?

1997 • 172 citations

Neurofibrillary axonal swellings and amyotrophic lateral sclerosis

1984 • 164 citations

Proteasome inhibition enhances the stability of mouse CuZn superoxide dismutase with mutations linked to familial amyotrophic lateral sclerosis

1996 • 118 citations

Molecular genetic basis of familial ALS

1996 • 97 citations

Breakdown of oxidized proteins as a part of secondary antioxidant defenses in mammalian cells

1997 • 95 citations

Intermediate filaments and ubiquitin: a new thread in the understanding of chronic neurodegenerative diseases.

1989 • 57 citations

Fragmentation of the Golgi apparatus of motor neurons in amyotrophic lateral sclerosis (ALS). Clinical studies in ALS of Guam and experimental studies in deafferented neurons and in beta,beta'-iminodipropionitrile axonopathy.

1994 • 57 citations

Ultrastructure of multiple microtubule initiation sites in mouse neuroblastoma cells

1981 • 45 citations

Presence of Cu/Zn superoxide dismutase (SOD) immunoreactivity in neuronal hyaline inclusions in spinal cords from mice carrying a transgene for Gly93Ala mutant human Cu/Zn SOD

1998 • 44 citations

Formation of granular cytoplasmic aggregates in COS7 cells expressing mutant Cu/Zn superoxide dismutase associated with familial amyotrophic lateral sclerosis

1998 • 39 citations

Neurofibrillary tangles in progressive supranuclear palsy brains exhibit immunoreactivity to frameshift mutant ubiquitin-B protein

2000 • 31 citations

Superoxide dismutase and neurofilament transgenic models of amyotrophic lateral sclerosis

1998 • 27 citations

Molecular genetics of familial amyotrophic lateral sclerosis.

1991 • 25 citations

Morphological Aspects of Superoxide Dismutase-1 Mutation in Amyotrophic Lateral Sclerosis and its Transgenic Mouse Model.

1999 • 7 citations