(Q41235562)

28 September 2017

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title

Transgenic models of neurodegenerative diseases (English)

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28 September 2017

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1

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28 September 2017

Borchelt DR

2

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28 September 2017

Wong PC

series ordinal

3

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28 September 2017

Sisodia SS

series ordinal

4

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28 September 2017

Price DL

series ordinal

5

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28 September 2017

1 October 1996

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Current Opinion in Neurobiology

28 September 2017

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28 September 2017

6

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28 September 2017

651-660

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28 September 2017

5

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

28 September 2017

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Alzheimer-type neuropathology in transgenic mice overexpressing V717F β-amyloid precursor protein

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Spontaneous neurodegeneration in transgenic mice with mutant prion protein

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

SCA1 transgenic mice: A model for neurodegeneration caused by an expanded CAG trinucleotide repeat

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Linkage analysis in familial amyotrophic lateral sclerosis

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Linkage of a gene causing familial amyotrophic lateral sclerosis to chromosome 21 and evidence of genetic-locus heterogeneity

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Two novel mutations in the gene for copper zinc superoxide dismutase in UK families with amyotrophic lateral sclerosis.

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Oxygen radicals as key mediators in neurological disease: fact or fiction?

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Protein oxidation and aging

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Apparent hydroxyl radical production by peroxynitrite: implications for endothelial injury from nitric oxide and superoxide

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Marked increase in nitric oxide synthase mRNA in rat dorsal root ganglia after peripheral axotomy: in situ hybridization and functional studies

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

A redox-based mechanism for the neuroprotective and neurodestructive effects of nitric oxide and related nitroso-compounds.

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Cu/Zn superoxide dismutase activity in familial and sporadic amyotrophic lateral sclerosis

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Extension of life-span by overexpression of superoxide dismutase and catalase in Drosophila melanogaster.

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Phenotypic rescue by a bovine transgene in a Cu/Zn superoxide dismutase-null mutant of Drosophila melanogaster

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Chronic inhibition of superoxide dismutase produces apoptotic death of spinal neurons

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Mutations associated with amyotrophic lateral sclerosis convert superoxide dismutase from an antiapoptotic gene to a proapoptotic gene: studies in yeast and neural cells

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Toxic mutants in Charcot's sclerosis

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Altered reactivity of superoxide dismutase in familial amyotrophic lateral sclerosis

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Analysis of the KSP repeat of the neurofilament heavy subunit in familiar amyotrophic lateral sclerosis

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Spinal and bulbar muscular atrophy: a trinucleotide-repeat expansion neurodegenerative disease

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Identification and characterization of a spinal muscular atrophy-determining gene

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

The gene for neuronal apoptosis inhibitory protein is partially deleted in individuals with spinal muscular atrophy

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

ALS, SOD and peroxynitrite

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Superoxide dismutase is an abundant component in cell bodies, dendrites, and axons of motor neurons and in a subset of other neurons

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

The requirement for yeast superoxide dismutase is bypassed through mutations in BSD2, a novel metal homeostasis gene

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Mutations in PMR1 suppress oxidative damage in yeast cells lacking superoxide dismutase

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Motor neuron disease caused by mutations in superoxide dismutase 1.

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Changes in neurofilament transport coincide temporally with alterations in the caliber of axons in regenerating motor fibers

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Neurofilament gene expression: a major determinant of axonal caliber

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Neurofilament-deficient axons and perikaryal aggregates in viable transgenic mice expressing a neurofilament-beta-galactosidase fusion protein

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Proximal axonal enlargement in motor neuron disease.

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Neurofilamentous axonal swellings as a normal finding in the spinal anterior horn of man and other primates

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Slow Axonal Transport of Neurofilament Proteins: Impairment of β,β′-Iminodipropionitrile Administration

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Incidence of dementia and probable Alzheimer's disease in a general population: the Framingham Study.

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

The US economic and social costs of Alzheimer's disease revisited

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Genetic evidence for a novel familial Alzheimer's disease locus on chromosome 14.

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

The structure of the presenilin 1 (S182) gene and identification of six novel mutations in early onset AD families

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Cloning of a gene bearing missense mutations in early-onset familial Alzheimer's disease

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Familial Alzheimer's chromosome 14 mutations.

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Candidate gene for the chromosome 1 familial Alzheimer's disease locus

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

A familial Alzheimer's disease locus on chromosome 1.

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Familial Alzheimer's disease in kindreds with missense mutations in a gene on chromosome 1 related to the Alzheimer's disease type 3 gene

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Early-onset Alzheimer's disease caused by mutations at codon 717 of the beta-amyloid precursor protein gene

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Segregation of a missense mutation in the amyloid precursor protein gene with familial Alzheimer's disease

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Presenile dementia and cerebral haemorrhage linked to a mutation at codon 692 of the beta-amyloid precursor protein gene.

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

A pathogenic mutation for probable Alzheimer's disease in the APP gene at the N-terminus of beta-amyloid

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Gene dose of apolipoprotein E type 4 allele and the risk of Alzheimer's disease in late onset families

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Apolipoprotein E affects the rate of Alzheimer disease expression: beta-amyloid burden is a secondary consequence dependent on APOE genotype and duration of disease

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Endoproteolysis of presenilin 1 and accumulation of processed derivatives in vivo

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Secreted amyloid beta-protein similar to that in the senile plaques of Alzheimer's disease is increased in vivo by the presenilin 1 and 2 and APP mutations linked to familial Alzheimer's disease

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Mutation of the Alzheimer's disease amyloid gene in hereditary cerebral hemorrhage, Dutch type.

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Amyloid beta protein precursor gene and hereditary cerebral hemorrhage with amyloidosis (Dutch)

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Mutation of the beta-amyloid precursor protein in familial Alzheimer's disease increases beta-protein production

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Release of excess amyloid beta protein from a mutant amyloid beta protein precursor.

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Metabolism of the "Swedish" amyloid precursor protein variant in neuro2a (N2a) cells. Evidence that cleavage at the "beta-secretase" site occurs in the golgi apparatus

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Peptides homologous to the amyloid protein of Alzheimer's disease containing a glutamine for glutamic acid substitution have accelerated amyloid fibril formation.

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

An increased percentage of long amyloid beta protein secreted by familial amyloid beta protein precursor (beta APP717) mutants.

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Seeding "one-dimensional crystallization" of amyloid: a pathogenic mechanism in Alzheimer's disease and scrapie?

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

The carboxy terminus of the beta amyloid protein is critical for the seeding of amyloid formation: implications for the pathogenesis of Alzheimer's disease

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Visualization of A beta 42(43) and A beta 40 in senile plaques with end-specific A beta monoclonals: evidence that an initially deposited species is A beta 42(43).

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Sequence of deposition of heterogeneous amyloid beta-peptides and APO E in Down syndrome: implications for initial events in amyloid plaque formation.

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

The apolipoprotein epsilon 4 allele in patients with Alzheimer's disease

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Apolipoprotein E polymorphism and Alzheimer's disease.

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Apolipoprotein E in sporadic Alzheimer's disease: allelic variation and receptor interactions

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Association of apolipoprotein E allele epsilon 4 with late-onset familial and sporadic Alzheimer's disease

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Apolipoprotein E: high-avidity binding to beta-amyloid and increased frequency of type 4 allele in late-onset familial Alzheimer disease

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Apolipoprotein E, epsilon 4 allele as a major risk factor for sporadic early and late-onset forms of Alzheimer's disease: analysis of the 19q13.2 chromosomal region.

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Apolipoprotein E genotypes and Alzheimer's disease in a community study of elderly African Americans.

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Cellular and molecular biology of Alzheimer's disease and animal models

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Levels and alternative splicing of amyloid beta protein precursor (APP) transcripts in brains of APP transgenic mice and humans with Alzheimer's disease.

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

1 reference

7 January 2021

Scrapie and cellular prion proteins differ in their kinetics of synthesis and topology in cultured cells

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Conversion of alpha-helices into beta-sheets features in the formation of the scrapie prion proteins

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Structural studies of the scrapie prion protein using mass spectrometry and amino acid sequencing.

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

NMR structure of the mouse prion protein domain PrP(121-231)

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Serial transmission in rodents of neurodegeneration from transgenic mice expressing mutant prion protein

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Prion propagation in mice expressing human and chimeric PrP transgenes implicates the interaction of cellular PrP with another protein

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

The expanding world of trinucleotide repeats

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Polyglutamine expansion as a pathological epitope in Huntington's disease and four dominant cerebellar ataxias

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Machado-Joseph disease and SCA3: the genotype meets the phenotypes

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Neurobiology of Huntington's disease

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Friedreich's ataxia: autosomal recessive disease caused by an intronic GAA triplet repeat expansion

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Normal and expanded Huntington's disease gene alleles produce distinguishable proteins due to translation across the CAG repeat.

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Stability of an expanded trinucleotide repeat in the androgen receptor gene in transgenic mice

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Incorporation of glutamine repeats makes protein oligomerize: implications for neurodegenerative diseases

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

A huntingtin-associated protein enriched in brain with implications for pathology

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

Huntingtin and DRPLA proteins selectively interact with the enzyme GAPDH.

1 reference

https://api.crossref.org/works/10.1016%2FS0959-4388%2896%2980099-7

7 January 2021

10.1016/S0959-4388(96)80099-7

Identifiers

DOI

1 reference

28 September 2017

1 reference