Testosterone reduction prevents phenotypic expression in a transgenic mouse model of spinal and bulbar muscular atrophy (Q44173664)

30 November 2017

title

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

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30 November 2017

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2

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30 November 2017

10

Gen Sobue

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30 November 2017

Masahisa Katsuno

1

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30 November 2017

Akito Kume

3

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30 November 2017

Mei Li

4

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30 November 2017

Yuji Nakagomi

5

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30 November 2017

Hisayoshi Niwa

6

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30 November 2017

Chen Sang

7

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30 November 2017

Yasushi Kobayashi

8

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30 November 2017

Manabu Doyu

9

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30 November 2017

language of work or name

English

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publication date

1 August 2002

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30 November 2017

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30 November 2017

volume

35

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30 November 2017

issue

5

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30 November 2017

page(s)

843-854

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

30 November 2017

cites work

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Creatine increase survival and delays motor symptoms in a transgenic animal model of Huntington's disease

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

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The length and location of CAG trinucleotide repeats in the androgen receptor N-terminal domain affect transactivation function

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

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

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

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

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Trinucleotide repeat length instability and age of onset in Huntington's disease

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

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

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Strong correlation between the number of CAG repeats in androgen receptor genes and the clinical onset of features of spinal and bulbar muscular atrophy

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Evidence for a repressive function of the long polyglutamine tract in the human androgen receptor: possible pathogenetic relevance for the (CAG)n-expanded neuronopathies

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

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7 January 2021

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

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7 January 2021

Androgen receptor YAC transgenic mice carrying CAG 45 alleles show trinucleotide repeat instability.

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7 January 2021

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

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7 January 2021

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Defects of androgen receptor function: from sex reversal to motor neurone disease

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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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Tissue-specific proteolysis of Huntingtin (htt) in human brain: evidence of enhanced levels of N- and C-terminal htt fragments in Huntington's disease striatum.

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

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7 January 2021

Transcriptional activation by the androgen receptor in X-linked spinal and bulbar muscular atrophy

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7 January 2021

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

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7 January 2021

Efficient selection for high-expression transfectants with a novel eukaryotic vector

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7 January 2021

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

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7 January 2021

Inhibition of caspase-1 slows disease progression in a mouse model of Huntington's disease

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Nuclear targeting of mutant Huntingtin increases toxicity

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7 January 2021

Lessons from animal models of Huntington's disease

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7 January 2021

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

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7 January 2021

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

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7 January 2021

X-linked recessive bulbospinal neuronopathy. A clinicopathological study

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7 January 2021

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

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7 January 2021

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

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7 January 2021

Histone deacetylase inhibitors arrest polyglutamine-dependent neurodegeneration in Drosophila

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7 January 2021

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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7 January 2021

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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7 January 2021

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7 January 2021

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

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7 January 2021

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

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7 January 2021

Caspase cleavage of gene products associated with triplet expansion disorders generates truncated fragments containing the polyglutamine tract

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7 January 2021

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

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7 January 2021

Glutamine repeats and neurodegeneration

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7 January 2021

10.1016/S0896-6273(02)00834-6

Identifiers

DOI

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30 November 2017

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