(Q35767605)

10 August 2017

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title

Spinal and bulbar muscular atrophy: ligand-dependent pathogenesis and therapeutic perspectives (English)

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10 August 2017

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4

Gen Sobue

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10 August 2017

Masahisa Katsuno

1

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PubMed ID

15133611

10 August 2017

Hiroaki Adachi

series ordinal

2

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PubMed ID

15133611

10 August 2017

Fumiaki Tanaka

series ordinal

3

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10 August 2017

27 February 2004

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Journal of Molecular Medicine

10 August 2017

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10 August 2017

82

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10 August 2017

5

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10 August 2017

298-307

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https://scigraph.springernature.com/pub.10.1007/s00109-004-0530-7

10 August 2017

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cites work

Altered transcriptional regulation in cells expressing the expanded polyglutamine androgen receptor

1 reference

12 December 2020

Nucleocytoplasmic transport signals affect the age at onset of abnormalities in knock-in mice expressing polyglutamine within an ectopic protein context

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12 December 2020

Histone deacetylase inhibitors arrest polyglutamine-dependent neurodegeneration in Drosophila

1 reference

12 December 2020

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

1 reference

12 December 2020

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

1 reference

12 December 2020

Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain

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12 December 2020

Hiroshi Kawahara (1858-1918).

1 reference

12 December 2020

Familial bulbo-spinal muscular atrophy associated with testicular atrophy and sensory neuropathy (Kennedy-Alter-Sung syndrome). Autopsy case report of two brothers

1 reference

12 December 2020

Pivotal role of oligomerization in expanded polyglutamine neurodegenerative disorders.

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12 December 2020

Interference by huntingtin and atrophin-1 with cbp-mediated transcription leading to cellular toxicity

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12 December 2020

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

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12 December 2020

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

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12 December 2020

Over-expression of inducible HSP70 chaperone suppresses neuropathology and improves motor function in SCA1 mice

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12 December 2020

Glutamine repeats and neurodegeneration

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12 December 2020

Reversal of neuropathology and motor dysfunction in a conditional model of Huntington's disease

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12 December 2020

Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice

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12 December 2020

CAG-repeat expansion in androgen receptor in Kennedy's disease is not a loss of function mutation

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12 December 2020

Expanded polyglutamine in the Machado-Joseph disease protein induces cell death in vitro and in vivo

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12 December 2020

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

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12 December 2020

Tissue-specific somatic mosaicism in spinal and bulbar muscular atrophy is dependent on CAG-repeat length and androgen receptor--gene expression level.

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12 December 2020

HEREDITARY PROXIMAL NEUROGENIC MUSCULAR ATROPHY IN ADULT

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12 December 2020

Sweet relief for Huntington disease

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12 December 2020

Ligand-dependent aggregation of polyglutamine-expanded androgen receptor in neuronal cells

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12 December 2020

CREB-binding protein sequestration by expanded polyglutamine

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12 December 2020

Transglutaminase potentiates ligand-dependent proteasome dysfunction induced by polyglutamine-expanded androgen receptor

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12 December 2020

Proximal spinal muscular atrophy

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12 December 2020

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

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12 December 2020

Caspase-3 cleaves the expanded androgen receptor protein of spinal and bulbar muscular atrophy in a polyglutamine repeat length-dependent manner

1 reference

12 December 2020

Decrease in androgen binding and effect of androgen treatment in a case of X-linked bulbospinal neuronopathy

1 reference

12 December 2020

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

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12 December 2020

Familial progressive bulbar‐spinal muscular atrophy

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12 December 2020

X-linked bulbospinal neuronopathy: Kennedy disease

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12 December 2020

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

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12 December 2020

Serine 776 of ataxin-1 is critical for polyglutamine-induced disease in SCA1 transgenic mice

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12 December 2020

Expansion explosion: new clues to the pathogenesis of repeat expansion neurodegenerative diseases

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12 December 2020

Androgen insensitivity.

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12 December 2020

Interaction of Akt-phosphorylated ataxin-1 with 14-3-3 mediates neurodegeneration in spinocerebellar ataxia type 1.

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12 December 2020

High prevalence of Kennedy's disease in Western Finland -- is the syndrome underdiagnosed?

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12 December 2020

Neuroprotective effects of gonadal steroids on regenerating peripheral motoneurons

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12 December 2020

Phenotypic manifestations associated with CAG-repeat expansion in the androgen receptor gene in male patients and heterozygous females: a clinical and molecular study of 30 families

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12 December 2020

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

1 reference

12 December 2020

Central motor and sensory conduction in X-linked recessive bulbospinal neuronopathy

1 reference

12 December 2020

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

1 reference

12 December 2020

Androgen receptor mRNA with increased size of tandem CAG repeat is widely expressed in the neural and nonneural tissues of X-linked recessive bulbospinal neuronopathy

1 reference

12 December 2020

A comprehensive endocrine description of Kennedy's disease revealing androgen insensitivity linked to CAG repeat length

1 reference

12 December 2020

Suppression of aggregate formation and apoptosis by transglutaminase inhibitors in cells expressing truncated DRPLA protein with an expanded polyglutamine stretch.

1 reference

12 December 2020

A cell culture model for androgen effects in motor neurons

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12 December 2020

Transglutaminase aggregates huntingtin into nonamyloidogenic polymers, and its enzymatic activity increases in Huntington's disease brain nuclei

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12 December 2020

Thirty-seven CAG repeats in the androgen receptor gene in two healthy individuals

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12 December 2020

Huntington's disease: the challenge for cell biologists

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12 December 2020

Epidemiologic investigations of amyotrophic lateral sclerosis. III. A genetic interpretation of incidence and geographic distribution

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12 December 2020

Founder effect in spinal and bulbar muscular atrophy (SBMA)

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12 December 2020

Chaperones Hsp70 and Hsp40 suppress aggregate formation and apoptosis in cultured neuronal cells expressing truncated androgen receptor protein with expanded polyglutamine tract

1 reference

12 December 2020

Subclinical phenotypic expressions in heterozygous females of X-linked recessive bulbospinal neuronopathy

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12 December 2020

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

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12 December 2020

Polyglutamine pathogenesis: emergence of unifying mechanisms for Huntington's disease and related disorders

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12 December 2020

Specificity of ligand-dependent androgen receptor stabilization: receptor domain interactions influence ligand dissociation and receptor stability

1 reference

12 December 2020

Progressive proximal spinal and bulbar muscular atrophy of late onset. A sex-linked recessive trait

1 reference

12 December 2020

Aggresomes protect cells by enhancing the degradation of toxic polyglutamine-containing protein

1 reference

12 December 2020

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

1 reference

12 December 2020

THE WOHLFART-KUGELBERG-WELANDER DISEASE; REVIEW OF THE LITERATURE AND REPORT OF A CASE.

1 reference

12 December 2020

Expression of X-linked bulbospinal muscular atrophy (Kennedy disease) in two homozygous women

1 reference

12 December 2020

Nuclear inclusions of the androgen receptor protein in spinal and bulbar muscular atrophy.

1 reference

12 December 2020

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

1 reference

12 December 2020

Severity of X-linked recessive bulbospinal neuronopathy correlates with size of the tandem CAG repeat in androgen receptor gene

1 reference

12 December 2020

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

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12 December 2020

Reduced transcriptional regulatory competence of the androgen receptor in X-linked spinal and bulbar muscular atrophy

1 reference

12 December 2020

Inhibition of huntingtin fibrillogenesis by specific antibodies and small molecules: implications for Huntington's disease therapy

1 reference

12 December 2020

Neural androgen receptor regulation: effects of androgen and antiandrogen

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12 December 2020

Heat shock protein 70 chaperone overexpression ameliorates phenotypes of the spinal and bulbar muscular atrophy transgenic mouse model by reducing nuclear-localized mutant androgen receptor protein

1 reference

12 December 2020

Hormone-dependent androgen receptor phosphorylation is accompanied by receptor transformation in human lymph node carcinoma of the prostate cells

1 reference

12 December 2020

Suppression of polyglutamine-mediated neurodegeneration in Drosophila by the molecular chaperone HSP70.

1 reference

12 December 2020

Testosterone therapy and the pathogenesis of Kennedy's disease (X-linked bulbospinal muscular atrophy)

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12 December 2020

A family with adult spinal and bulbar muscular atrophy, X-linked inheritance and associated testicular failure

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12 December 2020

The subnuclear three-dimensional image analysis of androgen receptor fused to green fluorescence protein

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12 December 2020

Genetic modulation of polyglutamine toxicity by protein conjugation pathways in Drosophila

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12 December 2020

Toxic proteins in neurodegenerative disease

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12 December 2020

Multiple founder effects in spinal and bulbar muscular atrophy (SBMA, Kennedy disease) around the world

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12 December 2020

Spinal and cranial motor nerve roots in amyotrophic lateral sclerosis and X-linked recessive bulbospinal muscular atrophy: morphometric and teased-fiber study

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12 December 2020

X-linked spinal muscular atrophy (Kennedy's syndrome). A kindred with hypobetalipoproteinemia

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12 December 2020

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

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12 December 2020

Kennedy's disease. Phosphorylation of the polyglutamine-expanded form of androgen receptor regulates its cleavage by caspase-3 and enhances cell death

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12 December 2020

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12 December 2020

Protective effect of chaperones on polyglutamine diseases

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12 December 2020

Prolonged survival and decreased abnormal movements in transgenic model of Huntington disease, with administration of the transglutaminase inhibitor cystamine

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12 December 2020

A mouse model of spinal and bulbar muscular atrophy.

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12 December 2020

Bacterial and yeast chaperones reduce both aggregate formation and cell death in mammalian cell models of Huntington's disease

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12 December 2020

Spinobulbar muscular atrophy can mimic ALS: the importance of genetic testing in male patients with atypical ALS.

1 reference

12 December 2020

X-linked recessive bulbospinal neuronopathy: a report of ten cases

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12 December 2020

X-linked adult form of spinal muscular atrophy

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12 December 2020

Nuclear targeting of mutant Huntingtin increases toxicity

1 reference

12 December 2020

Androgen receptor phosphorylation, turnover, nuclear transport, and transcriptional activation. Specificity for steroids and antihormones

1 reference

12 December 2020

Meiotic stability and genotype-phenotype correlation of the trinucleotide repeat in X-linked spinal and bulbar muscular atrophy

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12 December 2020

Generation and characterization of androgen receptor knockout (ARKO) mice: an in vivo model for the study of androgen functions in selective tissues

1 reference

12 December 2020

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

1 reference

12 December 2020

Primary sensory neurons in X-linked recessive bulbospinal neuropathy: histopathology and androgen receptor gene expression

1 reference

12 December 2020

Histone deacetylase inhibitors reduce polyglutamine toxicity

1 reference

12 December 2020

10.1007/S00109-004-0530-7

Identifiers

DOI

1 reference

10 August 2017

1 reference

PubMed ID

15133611