(Q28206361)

7 January 2021

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

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7 January 2021

Fine structural study of neurofibrillary changes in a family with amyotrophic lateral sclerosis

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7 January 2021

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

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7 January 2021

Familial adult motor neuron disease: amyotrophic lateral sclerosis

1 reference

7 January 2021

Superoxide dismutase activity is essential for stationary phase survival in Saccharomyces cerevisiae. Mitochondrial production of toxic oxygen species in vivo

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7 January 2021

Current status of SOD1 mutations in familial amyotrophic lateral sclerosis

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7 January 2021

Genetics of sporadic ALS.

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7 January 2021

Amyotrophic lateral sclerosis associated with homozygosity for an Asp90Ala mutation in CuZn-superoxide dismutase

1 reference

7 January 2021

Autosomal recessive adult-onset amyotrophic lateral sclerosis associated with homozygosity for Asp90Ala CuZn-superoxide dismutase mutation. A clinical and genealogical study of 36 patients

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7 January 2021

Amyotrophic lateral sclerosis and structural defects in Cu,Zn superoxide dismutase

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7 January 2021

A novel SOD mutant and ALS

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7 January 2021

Motor neuron degeneration in mice that express a human Cu,Zn superoxide dismutase mutation

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7 January 2021

An adverse property of a familial ALS-linked SOD1 mutation causes motor neuron disease characterized by vacuolar degeneration of mitochondria

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7 January 2021

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

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7 January 2021

ALS-linked SOD1 mutant G85R mediates damage to astrocytes and promotes rapidly progressive disease with SOD1-containing inclusions.

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7 January 2021

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

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7 January 2021

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

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7 January 2021

Superoxide dismutase concentration and activity in familial amyotrophic lateral sclerosis

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7 January 2021

Toxic mutants in Charcot's sclerosis

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7 January 2021

Bcl-2: prolonging life in a transgenic mouse model of familial amyotrophic lateral sclerosis

1 reference

7 January 2021

Delaying caspase activation by Bcl-2: A clue to disease retardation in a transgenic mouse model of amyotrophic lateral sclerosis

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7 January 2021

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

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7 January 2021

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

1 reference

7 January 2021

Recruitment of the mitochondrial-dependent apoptotic pathway in amyotrophic lateral sclerosis

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7 January 2021

Chaperone-facilitated copper binding is a property common to several classes of familial amyotrophic lateral sclerosis-linked superoxide dismutase mutants

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7 January 2021

ALS, SOD and peroxynitrite

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7 January 2021

Increased 3-nitrotyrosine and oxidative damage in mice with a human copper/zinc superoxide dismutase mutation.

1 reference

7 January 2021

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 superox

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7 January 2021

Protein oxidative damage in a transgenic mouse model of familial amyotrophic lateral sclerosis

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7 January 2021

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

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7 January 2021

Human Cu/Zn superoxide dismutase (SOD1) overexpression in mice causes mitochondrial vacuolization, axonal degeneration, and premature motoneuron death and accelerates motoneuron disease in mice expressing a familial amyotrophic lateral sclerosis mut

1 reference

7 January 2021

Altered reactivity of superoxide dismutase in familial amyotrophic lateral sclerosis

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7 January 2021

Reexamination of the mechanism of hydroxyl radical adducts formed from the reaction between familial amyotrophic lateral sclerosis-associated Cu,Zn superoxide dismutase mutants and H2O2.

1 reference

7 January 2021

Superoxide dismutase 1 subunits with mutations linked to familial amyotrophic lateral sclerosis do not affect wild-type subunit function.

1 reference

7 January 2021

Decreased zinc affinity of amyotrophic lateral sclerosis-associated superoxide dismutase mutants leads to enhanced catalysis of tyrosine nitration by peroxynitrite

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7 January 2021

Induction of nitric oxide-dependent apoptosis in motor neurons by zinc-deficient superoxide dismutase

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7 January 2021

Toxicity of ALS-linked SOD1 mutants

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7 January 2021

Loss of in vitro metal ion binding specificity in mutant copper-zinc superoxide dismutases associated with familial amyotrophic lateral sclerosis

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7 January 2021

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

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7 January 2021

Nitrotyrosination contributes minimally to toxicity of mutant SOD1 associated with ALS.

1 reference

7 January 2021

Oxidative damage to protein in sporadic motor neuron disease spinal cord

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7 January 2021

Superoxide dismutase activity, oxidative damage, and mitochondrial energy metabolism in familial and sporadic amyotrophic lateral sclerosis

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7 January 2021

Massive mitochondrial degeneration in motor neurons triggers the onset of amyotrophic lateral sclerosis in mice expressing a mutant SOD1

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7 January 2021

The copper chaperone for superoxide dismutase

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7 January 2021

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

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7 January 2021

Evidence of increased oxidative damage in both sporadic and familial amyotrophic lateral sclerosis.

1 reference

7 January 2021

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

1 reference

7 January 2021

Histochemical and immunocytochemical study of ubiquitinated neuronal inclusions in amyotrophic lateral sclerosis

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7 January 2021

Ubiquitin-immunoreactive intraneuronal inclusions in amyotrophic lateral sclerosis. Morphology, distribution, and specificity.

1 reference

7 January 2021

Ubiquitin deposits in anterior horn cells in motor neurone disease.

1 reference

7 January 2021

Up-regulation of protein chaperones preserves viability of cells expressing toxic Cu/Zn-superoxide dismutase mutants associated with amyotrophic lateral sclerosis

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7 January 2021

Aggregation of mutant Cu/Zn superoxide dismutase proteins in a culture model of ALS

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7 January 2021

Knockout of glutamate transporters reveals a major role for astroglial transport in excitotoxicity and clearance of glutamate

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7 January 2021

Restricted expression of G86R Cu/Zn superoxide dismutase in astrocytes results in astrocytosis but does not cause motoneuron degeneration

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7 January 2021

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

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7 January 2021

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

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7 January 2021

Neuron-specific expression of mutant superoxide dismutase 1 in transgenic mice does not lead to motor impairment.

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7 January 2021

Proximal axonal enlargement in motor neuron disease.

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7 January 2021

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

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7 January 2021

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

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7 January 2021

Extra axonal neurofilaments do not exacerbate disease caused by mutant Cu,Zn superoxide dismutase.

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7 January 2021

Deregulation of Cdk5 in a mouse model of ALS: toxicity alleviated by perikaryal neurofilament inclusions

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7 January 2021

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

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7 January 2021

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

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7 January 2021

Modulations of neurofilament axonal transport during the development of rabbit retinal ganglion cells

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7 January 2021

Deletions of the heavy neurofilament subunit tail in amyotrophic lateral sclerosis

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7 January 2021

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

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7 January 2021

Novel insertion in the KSP region of the neurofilament heavy gene in amyotrophic lateral sclerosis (ALS)

1 reference

7 January 2021

Further evidence that neurofilament light chain gene mutations can cause Charcot-Marie-Tooth disease type 2E.

1 reference

7 January 2021

A new variant of Charcot-Marie-Tooth disease type 2 is probably the result of a mutation in the neurofilament-light gene

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7 January 2021

Subunit composition of neurofilaments specifies axonal diameter.

1 reference

7 January 2021

Morphometric Comparison of the Vulnerability of Peripheral Motor and Sensory Neurons in Amyotrophic Lateral Sclerosis

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7 January 2021

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

1 reference

7 January 2021

Epilepsy and exacerbation of brain injury in mice lacking the glutamate transporter GLT-1

1 reference

7 January 2021

Excitatory amino acids in amyotrophic lateral sclerosis: An update

1 reference

7 January 2021

Abnormal excitatory amino acid metabolism in amyotrophic lateral sclerosis

1 reference

7 January 2021

CSF and plasma amino acid levels in motor neuron disease: elevation of CSF glutamate in a subset of patients.

1 reference

7 January 2021

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

1 reference

7 January 2021

Aberrant RNA processing in a neurodegenerative disease: the cause for absent EAAT2, a glutamate transporter, in amyotrophic lateral sclerosis

1 reference

7 January 2021

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

1 reference

7 January 2021

Glutamate transporter EAAT2 splice variants occur not only in ALS, but also in AD and controls

1 reference

7 January 2021

Lathyrism: evidence for role of the neuroexcitatory aminoacid BOAA.

1 reference

7 January 2021

Guam ALS-PDC: possible causes

1 reference

7 January 2021

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

1 reference

7 January 2021

Autosomal dominant juvenile amyotrophic lateral sclerosis.

1 reference

7 January 2021

Linkage of the gene for an autosomal dominant form of juvenile amyotrophic lateral sclerosis to chromosome 9q34.

1 reference

7 January 2021

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

1 reference

7 January 2021

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

1 reference

7 January 2021

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

1 reference

7 January 2021

Parvalbumin and calbindin D-28k in the human motor system and in motor neuron disease.

1 reference

7 January 2021

Recessive amyotrophic lateral sclerosis families with the D90A SOD1 mutation share a common founder: evidence for a linked protective factor

1 reference

7 January 2021

Genetic mapping of a mouse modifier gene that can prevent ALS onset

1 reference

7 January 2021

A controlled trial of recombinant methionyl human BDNF in ALS: The BDNF Study Group (Phase III)

1 reference

7 January 2021

Expression of glial glutamate transporters GLT-1 and GLAST is unchanged in the hippocampus in fully kindled rats

1 reference

7 January 2021

A placebo-controlled trial of insulin-like growth factor-I in amyotrophic lateral sclerosis. European ALS/IGF-I Study Group

1 reference

7 January 2021

Effect of recombinant human insulin-like growth factor-I on progression of ALS. A placebo-controlled study. The North America ALS/IGF-I Study Group

1 reference

7 January 2021

A controlled trial of riluzole in amyotrophic lateral sclerosis. ALS/Riluzole Study Group

1 reference

7 January 2021

Dose-ranging study of riluzole in amyotrophic lateral sclerosis. Amyotrophic Lateral Sclerosis/Riluzole Study Group II

1 reference

7 January 2021

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

1 reference

7 January 2021

Increases in cortical glutamate concentrations in transgenic amyotrophic lateral sclerosis mice are attenuated by creatine supplementation.

1 reference

7 January 2021

Mutant Cu/Zn-superoxide dismutase proteins have altered solubility and interact with heat shock/stress proteins in models of amyotrophic lateral sclerosis

1 reference

7 January 2021

Linkage of familial amyotrophic lateral sclerosis with frontotemporal dementia to chromosome 9q21-q22

1 reference

7 January 2021

Synthetic superoxide dismutase/catalase mimetics reduce oxidative stress and prolong survival in a mouse amyotrophic lateral sclerosis model

1 reference

7 January 2021

Therapeutic benefits of putrescine-modified catalase in a transgenic mouse model of familial amyotrophic lateral sclerosis.

1 reference

7 January 2021

Effects of an inhibitor of poly(ADP-ribose) polymerase, desmethylselegiline, trientine, and lipoic acid in transgenic ALS mice.

1 reference

7 January 2021

Beneficial effect of ginseng root in SOD-1 (G93A) transgenic mice

1 reference

7 January 2021

N-acetyl-L-cysteine improves survival and preserves motor performance in an animal model of familial amyotrophic lateral sclerosis

1 reference

7 January 2021

Mice with a partial deficiency of manganese superoxide dismutase show increased vulnerability to the mitochondrial toxins malonate, 3-nitropropionic acid, and MPTP.

1 reference

7 January 2021

A specific inhibitor of janus kinase-3 increases survival in a transgenic mouse model of amyotrophic lateral sclerosis

1 reference

7 January 2021

Administration of nitric oxide synthase inhibitors does not alter disease course of amyotrophic lateral sclerosis SOD1 mutant transgenic mice

1 reference

7 January 2021

Benefit of a combined treatment with trientine and ascorbate in familial amyotrophic lateral sclerosis model mice

1 reference

7 January 2021

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

1 reference

7 January 2021

Carboxyfullerenes as neuroprotective agents.

1 reference

7 January 2021

A double-blind, placebo-controlled randomized clinical trial of alpha-tocopherol (vitamin E) in the treatment of amyotrophic lateral sclerosis. ALS riluzole-tocopherol Study Group

1 reference

7 January 2021

The copper chelator d-penicillamine delays onset of disease and extends survival in a transgenic mouse model of familial amyotrophic lateral sclerosis

1 reference

7 January 2021

Intrathecal cyclosporin prolongs survival of late-stage ALS mice

1 reference

7 January 2021

Beneficial effects of lysine acetylsalicylate, a soluble salt of aspirin, on motor performance in a transgenic model of amyotrophic lateral sclerosis.

1 reference

7 January 2021

A double-blind study of the effectiveness of cyclosporine in amyotrophic lateral sclerosis

1 reference

7 January 2021

Intrathecal administration of natural human interferon alpha in amyotrophic lateral sclerosis

1 reference

7 January 2021

Therapeutic plasmapheresis and plasma exchange

1 reference

7 January 2021

Ganglioside therapy for amyotrophic lateral sclerosis: a double-blind controlled trial.

1 reference

7 January 2021

A double-blind controlled trial of bovine brain gangliosides in amyotrophic lateral sclerosis.

1 reference

7 January 2021

Trials of ganglioside therapy for amyotrophic lateral sclerosis and diabetic neuropathy

1 reference

7 January 2021

Double-Blind Controlled Trial of Purified Brain Gangliosides in Amyotrophic Lateral Sclerosis and Experience with Peripheral Neuropathies

1 reference

7 January 2021

Inhibition of ICE slows ALS in mice

1 reference

7 January 2021

Motor neuron cell death in a mouse model of FALS is not mediated by the p53 cell survival regulator.

1 reference

7 January 2021

A clinical trial of verapamil in amyotrophic lateral sclerosis

1 reference

7 January 2021

Controlled trial of nimodipine in amyotrophic lateral sclerosis.

1 reference

7 January 2021

Pilot trial of branched-chain aminoacids in amyotrophic lateral sclerosis

1 reference

7 January 2021

Intramuscular grafts of myoblasts genetically modified to secrete glial cell line-derived neurotrophic factor prevent motoneuron loss and disease progression in a mouse model of familial amyotrophic lateral sclerosis

1 reference

7 January 2021

Protective effects of cardiotrophin-1 adenoviral gene transfer on neuromuscular degeneration in transgenic ALS mice

1 reference

7 January 2021

Intrathecal thyrotropin-releasing hormone does not alter the progressive course of ALS: experience with an intrathecal drug delivery system

1 reference

7 January 2021

Intravenous thyrotropin-releasing hormone in patients with amyotrophic lateral sclerosis. Dose-response and randomized concurrent placebo-controlled pilot studies

1 reference

7 January 2021

A double-blind, placebo-controlled trial of TRH in amyotrophic lateral sclerosis

1 reference

7 January 2021

Genistein is neuroprotective in murine models of familial amyotrophic lateral sclerosis and stroke.

1 reference

7 January 2021

Trial of octacosanol in amyotrophic lateral sclerosis

1 reference

7 January 2021

Amantadine and guanidine are ineffective in ALS

1 reference

7 January 2021

Transfer factor is ineffective in amyotrophic lateral sclerosis

1 reference

7 January 2021

Therapeutic trial of tilorone in ALS: Lack of benefit in a double-blind, placebo-controlled study

1 reference

7 January 2021

The use of isoprinosine in patients with amyotrophic lateral sclerosis

1 reference

7 January 2021

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

1 reference

7 January 2021

Identifiers

DOI

10.1038/35097565

1 reference

Consolidated OpenCitations Corpus – April 2017

Dimensions Publication ID

1319607

1032464423

0 references

1319607

1 reference

Consolidated OpenCitations Corpus – April 2017

Consolidated OpenCitations Corpus – April 2017

1 reference