Superoxide dismutase in familial amyotrophic lateral sclerosis: models for gain of function

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

1993 • 6,762 citations

Identification and inhibition of the ICE/CED-3 protease necessary for mammalian apoptosis

1995 • 4,003 citations

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

1994 • 3,959 citations

The C. elegans cell death gene ced-3 encodes a protein similar to mammalian interleukin-1β-converting enzyme

1993 • 2,484 citations

Yama/CPP32β, a mammalian homolog of CED-3, is a CrmA-inhibitable protease that cleaves the death substrate poly(ADP-ribose) polymerase

1995 • 2,402 citations

Peroxynitrite-mediated tyrosine nitration catalyzed by superoxide dismutase

1992 • 1,499 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

Altered gene expression in neurons during programmed cell death: identification of c-jun as necessary for neuronal apoptosis.

1994 • 820 citations

Superoxide dismutase delays neuronal apoptosis: A role for reactive oxygen species in programmed neuronal death

1995 • 744 citations

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

1995 • 694 citations

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

1993 • 627 citations

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

1994 • 604 citations

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

1993 • 549 citations

Superoxide Dismutases

1986 • 450 citations

Familial adult motor neuron disease: amyotrophic lateralsclerosis

1986 • 401 citations

Amyotrophic lateral sclerosis: Part 1. Clinical features, pathology, and ethical issues in management

1985 • 369 citations

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

1994 • 360 citations

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

1994 • 356 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

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

1994 • 291 citations

AMPA receptor activation potentiates zinc neurotoxicity

1993 • 280 citations

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

1994 • 271 citations

Enzyme function of copper, zinc superoxide dismutase as a free radical generator.

1993 • 224 citations

Zinc toxicity on cultured cortical neurons: Involvement of N-methyl-d-aspartate receptors

1994 • 223 citations

Rapid Communication: Cu/Zn Superoxide Dismutase Activity in Familial and Sporadic Amyotrophic Lateral Sclerosis

1994 • 161 citations

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

1995 • 149 citations

superoxide dismutase-like immunoreactivity in Lewy body-like inclusions of sporadic amyotrophic lateral sclerosis

1994 • 149 citations

Amyotrophic leteral sclerosis: Part 2. Etiopathogenesis

1985 • 146 citations

Superoxide Dismutase Concentration and Activity in Familial Amyotrophic Lateral Sclerosis

1995 • 125 citations

Cytotoxicity of immunoglobulins from amyotrophic lateral sclerosis patients on a hybrid motoneuron cell line.

1994 • 115 citations

Brainstem motoneuron pools that are selectively resistant in amyotrophic lateral sclerosis are preferentially enriched in parvalbumin: Evidence from monkey brainstem for a calcium-mediated mechanism in sporadic ALS

1995 • 114 citations

Identification of new mutations in the Cu/Zn superoxide dismutase gene of patients with familial amyotrophic lateral sclerosis.

1995 • 109 citations

Subunit-destabilizing mutations in Drosophila copper/zinc superoxide dismutase: neuropathology and a model of dimer dysequilibrium.

1995 • 107 citations

Characterization of three yeast copper-zinc superoxide dismutase mutants analogous to those coded for in familial amyotrophic lateral sclerosis.

1994 • 98 citations

Impaired copper binding by the H46R mutant of human Cu,Zn superoxide dismutase, involved in amyotrophic lateral sclerosis

1994 • 77 citations

A two basepair deletion in the SOD 1 gene causes familial amyotrophic lateral sclerosis.

1994 • 66 citations

Identification of three novel mutations in the gene for CuZn superoxide dismutase in patients with familial amyotrophic lateral sclerosis

1995 • 58 citations

IMMUNOHISTOCHEMICAL DEMONSTRATION OF Cu/Zn SUPEROXIDE DISMUTASE IN THE SPINAL CORD OF PATIENTS WITH FAMILIAL AMYOTRO-PHIC LATERAL SCLEROSIS

1993 • 51 citations

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

1994 • 42 citations

Determination of Superoxide Dismutase Activity by the Polarographic Method of Catalytic Currents in the Cerebrospinal Fluid of Aging Brain and Neurologic Degenerative Diseases

1991 • 36 citations

Red blood cell superoxide dismutase activity in sporadic amyotrophic lateral sclerosis

1994 • 18 citations

Indices of free radical activity in the cerebrospinal fluid in motor neuron disease.

1987 • 12 citations