(Q35592405)

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:14636160%20AND%20SRC:MED&resulttype=core&format=json

21 December 2019

review article

1 reference

title

Protein aggregation in motor neurone disorders (English)

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:14636160%20AND%20SRC:MED&resulttype=core&format=json

21 December 2019

1 reference

3

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:14636160%20AND%20SRC:MED&resulttype=core&format=json

21 December 2019

author name string

J D Wood

series ordinal

1

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:14636160%20AND%20SRC:MED&resulttype=core&format=json

21 December 2019

T P Beaujeux

series ordinal

2

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:14636160%20AND%20SRC:MED&resulttype=core&format=json

21 December 2019

publication date

1 December 2003

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:14636160%20AND%20SRC:MED&resulttype=core&format=json

Neuropathology and Applied Neurobiology

21 December 2019

published in

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:14636160%20AND%20SRC:MED&resulttype=core&format=json

21 December 2019

volume

29

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:14636160%20AND%20SRC:MED&resulttype=core&format=json

21 December 2019

issue

6

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:14636160%20AND%20SRC:MED&resulttype=core&format=json

21 December 2019

page(s)

529-545

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:14636160%20AND%20SRC:MED&resulttype=core&format=json

Spinobulbar muscular atrophy: polyglutamine-expanded androgen receptor is proteolytically resistant in vitro and processed abnormally in transfected cells.

21 December 2019

cites work

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

Expression of expanded repeat androgen receptor produces neurologic disease in transgenic mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

Transgenic mice with an expanded CAG repeat controlled by the human AR promoter show polyglutamine nuclear inclusions and neuronal dysfunction without neuronal cell death

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

Molecular chaperones enhance the degradation of expanded polyglutamine repeat androgen receptor in a cellular model of spinal and bulbar muscular atrophy

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

Cytoplasmic localization and the choice of ligand determine aggregate formation by androgen receptor with amplified polyglutamine stretch

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

Impairment of the ubiquitin-proteasome system by protein aggregation

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

A linear lattice model for polyglutamine in CAG-expansion diseases

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

Cell survival promoted by the Ras-MAPK signaling pathway by transcription-dependent and -independent mechanisms

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

Characterization of an expanded glutamine repeat androgen receptor in a neuronal cell culture system

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

Insights into Lou Gehrig's disease from the structure and instability of the A4V mutant of human Cu,Zn superoxide dismutase

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

From Charcot to SOD1: mechanisms of selective motor neuron death in ALS.

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

From Charcot to Lou Gehrig: deciphering selective motor neuron death in ALS

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1.

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

Mutation of the E6-AP ubiquitin ligase reduces nuclear inclusion frequency while accelerating polyglutamine-induced pathology in SCA1 mice.

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

Sp1 and TAFII130 transcriptional activity disrupted in early Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

Amyloid-like filaments and water-filled nanotubes formed by SOD1 mutant proteins linked to familial ALS

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

Identification of benzothiazoles as potential polyglutamine aggregation inhibitors of Huntington's disease by using an automated filter retardation assay

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

Familial amyotrophic lateral sclerosis. A subgroup characterized by posterior and spinocerebellar tract involvement and hyaline inclusions in the anterior horn cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

Suberoylanilide hydroxamic acid, a histone deacetylase inhibitor, ameliorates motor deficits in a mouse model of Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

A YAC mouse model for Huntington's disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration.

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

Hsp105alpha suppresses the aggregation of truncated androgen receptor with expanded CAG repeats and cell toxicity

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

Aggresomes: a cellular response to misfolded proteins

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

Protein precipitation: a common etiology in neurodegenerative disorders?

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

How do neurons die in neurodegenerative diseases?

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

Leuprorelin rescues polyglutamine-dependent phenotypes in a transgenic mouse model of spinal and bulbar muscular atrophy

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

Testosterone reduction prevents phenotypic expression in a transgenic mouse model of spinal and bulbar muscular atrophy

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

Ataxin-1 nuclear localization and aggregation: role in polyglutamine-induced disease in SCA1 transgenic mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

Aggregation of huntingtin in yeast varies with the length of the polyglutamine expansion and the expression of chaperone proteins

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

Androgen receptor gene mutations in X-linked spinal and bulbar muscular atrophy

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

No correlation between aggregates of Cu/Zn superoxide dismutase and cell death in familial amyotrophic lateral sclerosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

Amino-terminal fragments of mutant huntingtin show selective accumulation in striatal neurons and synaptic toxicity

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

Nonneural nuclear inclusions of androgen receptor protein in spinal and bulbar muscular atrophy

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

Common denominator of Cu/Zn superoxide dismutase mutants associated with amyotrophic lateral sclerosis: decreased stability of the apo state

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

Histone deacetylase inhibitors reduce polyglutamine toxicity

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

CREB-binding protein sequestration by expanded polyglutamine

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

A mouse model of spinal and bulbar muscular atrophy

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

Cleavage, aggregation and toxicity of the expanded androgen receptor in spinal and bulbar muscular atrophy

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

Dorfin ubiquitylates mutant SOD1 and prevents mutant SOD1-mediated neurotoxicity

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

Subcellular distribution of superoxide dismutases (SOD) in rat liver: Cu,Zn-SOD in mitochondria

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

Amyotrophic lateral sclerosis: a proposed mechanism

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

Ectopically Expressed CAG Repeats Cause Intranuclear Inclusions and a Progressive Late Onset Neurological Phenotype in the Mouse

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

Intranuclear inclusions of expanded polyglutamine protein in spinocerebellar ataxia type 3.

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

Nuclear targeting of mutant Huntingtin increases toxicity

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

Rubinstein-Taybi syndrome caused by mutations in the transcriptional co-activator CBP

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

Huntingtin spheroids and protofibrils as precursors in polyglutamine fibrilization

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

Oxidation-induced misfolding and aggregation of superoxide dismutase and its implications for amyotrophic lateral sclerosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

Familial amyotrophic lateral sclerosis-associated mutations decrease the thermal stability of distinctly metallated species of human copper/zinc superoxide dismutase

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions.

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

Huntingtin-encoded polyglutamine expansions form amyloid-like protein aggregates in vitro and in vivo

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

Self-assembly of polyglutamine-containing huntingtin fragments into amyloid-like fibrils: implications for Huntington's disease pathology

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

Riluzole prolongs survival time and alters nuclear inclusion formation in a transgenic mouse model of Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

Intranuclear inclusions and neuritic aggregates in transgenic mice expressing a mutant N-terminal fragment of huntingtin

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

Nuclear accumulation of truncated atrophin-1 fragments in a transgenic mouse model of DRPLA.

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

Intraneuronal conglomerates in sporadic motor neuron disease. A light and electron microscopic study

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

Expanded polyglutamine stretches interact with TAFII130, interfering with CREB-dependent transcription

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

Motoneuronal cell death is not correlated with aggregate formation of androgen receptors containing an elongated polyglutamine tract

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

Histone deacetylase inhibitors arrest polyglutamine-dependent neurodegeneration in Drosophila

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

The Huntington's disease protein interacts with p53 and CREB-binding protein and represses transcription

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

Polyglutamine-expanded androgen receptors form aggregates that sequester heat shock proteins, proteasome components and SRC-1, and are suppressed by the HDJ-2 chaperone

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

Hsp70 and Hsp40 improve neurite outgrowth and suppress intracytoplasmic aggregate formation in cultured neuronal cells expressing mutant SOD1.

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

Androgen-dependent neurodegeneration by polyglutamine-expanded human androgen receptor in Drosophila.

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

Abnormal skeletal patterning in embryos lacking a single Cbp allele: a partial similarity with Rubinstein-Taybi syndrome.

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

Aggregates in neurodegenerative disease: crowds and power?

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

Ligand promotes intranuclear inclusions in a novel cell model of spinal and bulbar muscular atrophy

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

High molecular weight complexes of mutant superoxide dismutase 1: age-dependent and tissue-specific accumulation

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

Fibrillar inclusions and motor neuron degeneration in transgenic mice expressing superoxide dismutase 1 with a disrupted copper-binding site

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

Histological evidence of protein aggregation in mutant SOD1 transgenic mice and in amyotrophic lateral sclerosis neural tissues

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

Bicarbonate-dependent peroxidase activity of human Cu,Zn-superoxide dismutase induces covalent aggregation of protein: intermediacy of tryptophan-derived oxidation products

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

Glutamine repeats and neurodegeneration

21 January 2018

1 reference

https://api.crossref.org/works/10.1046%2FJ.0305-1846.2003.00518.X

10.1046/J.0305-1846.2003.00518.X

21 January 2018

Identifiers

DOI

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:14636160%20AND%20SRC:MED&resulttype=core&format=json

21 December 2019

1 reference