Spinocerebellar ataxias caused by polyglutamine expansions: a review of therapeutic strategies (Q37140780)

18 August 2017

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title

Spinocerebellar ataxias caused by polyglutamine expansions: a review of therapeutic strategies (English)

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18 August 2017

Benjamin R Underwood

1

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PubMed ID

18418676

18 August 2017

David C Rubinsztein

series ordinal

2

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18 August 2017

1 January 2008

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18 August 2017

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18 August 2017

7

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18 August 2017

2

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18 August 2017

215-221

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https://scigraph.springernature.com/pub.10.1007/s12311-008-0026-z

18 August 2017

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cites work

Polyglutamine expansion down-regulates specific neuronal genes before pathologic changes in SCA1.

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Neuroprotective effects of phenylbutyrate in the N171-82Q transgenic mouse model of Huntington's disease

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Histone deacetylase inhibitors arrest polyglutamine-dependent neurodegeneration in Drosophila

21 January 2018

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Mice lacking ataxin-1 display learning deficits and decreased hippocampal paired-pulse facilitation

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Geldanamycin activates a heat shock response and inhibits huntingtin aggregation in a cell culture model of Huntington's disease

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Tetrahydrobiopterin double-blind, crossover trial in Machado-Joseph disease

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12 December 2020

Lithium induces autophagy by inhibiting inositol monophosphatase

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Effects of tandospirone on "5-HT1A receptor-associated symptoms" in patients with Machado-Josephe disease: an open-label study

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Cystamine inhibits caspase activity. Implications for the treatment of polyglutamine disorders

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Polyglutamine-expanded ataxin-3 activates mitochondrial apoptotic pathway by upregulating Bax and downregulating Bcl-xL.

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12 December 2020

Huntington disease patients and transgenic mice have similar pro-catabolic serum metabolite profiles

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12 December 2020

Inhibition of mTOR induces autophagy and reduces toxicity of polyglutamine expansions in fly and mouse models of Huntington disease

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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

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

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12 December 2020

Autosomal dominant cerebellar ataxia (SCA6) associated with small polyglutamine expansions in the alpha 1A-voltage-dependent calcium channel

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Caspase-mediated proteolysis of the polyglutamine disease protein ataxin-3.

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12 December 2020

Minocycline inhibits caspase-1 and caspase-3 expression and delays mortality in a transgenic mouse model of Huntington disease

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12 December 2020

Contrast sensitivity improvement with sulfamethoxazole and trimethoprim in a patient with Machado-Joseph disease without spasticity

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12 December 2020

Caspase-8 is required for cell death induced by expanded polyglutamine repeats.

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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

Polyglutamine-expanded ataxin-7 inhibits STAGA histone acetyltransferase activity to produce retinal degeneration

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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

NMDA-evoked excitotoxicity increases tissue transglutaminase in cerebellar granule cells.

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12 December 2020

A long CAG repeat in the mouse Sca1 locus replicates SCA1 features and reveals the impact of protein solubility on selective neurodegeneration.

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12 December 2020

Mechanisms of cell death in polyglutamine expansion diseases

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12 December 2020

Anterior cingulate cortical transplantation in transgenic Huntington's disease mice

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12 December 2020

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

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The therapeutic role of creatine in Huntington's disease

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12 December 2020

Therapeutic effects of coenzyme Q10 and remacemide in transgenic mouse models of Huntington's disease.

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12 December 2020

Evaluation of the effect of sulphametoxazole and trimethoprim in patients with Machado-Joseph disease

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12 December 2020

Intranuclear neuronal inclusions in Huntington's disease and dentatorubral and pallidoluysian atrophy: correlation between the density of inclusions and IT15 CAG triplet repeat length

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The polyglutamine neurodegenerative protein ataxin 3 regulates aggresome formation

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12 December 2020

Ectopically expressed CAG repeats cause intranuclear inclusions and a progressive late onset neurological phenotype in the mouse.

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RNAi suppresses polyglutamine-induced neurodegeneration in a model of spinocerebellar ataxia.

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Cerebellar allografts survive and transiently alleviate ataxia in a transgenic model of spinocerebellar ataxia type-1.

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Mutant huntingtin directly increases susceptibility of mitochondria to the calcium-induced permeability transition and cytochrome c release

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12 December 2020

Analysis of heat shock transcription factor for suppression of polyglutamine toxicity.

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12 December 2020

Allele-specific silencing of dominant disease genes

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12 December 2020

Oxidative stimuli affect polyglutamine aggregation and cell death in human mutant ataxin-1-expressing cells

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12 December 2020

Normal huntingtin function: an alternative approach to Huntington's disease

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12 December 2020

Histone Deacetylase Inhibition by Sodium Butyrate Chemotherapy Ameliorates the Neurodegenerative Phenotype in Huntington's Disease Mice

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12 December 2020

Hsp70 and Hsp40 attenuate formation of spherical and annular polyglutamine oligomers by partitioning monomer

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12 December 2020

Clinical and molecular events in patients with Machado-Joseph disease under lamotrigine therapy

1 reference

12 December 2020

Double-blind crossover trial of trimethoprim-sulfamethoxazole in spinocerebellar ataxia type 3/Machado-Joseph disease

1 reference

12 December 2020

Clinical trial of acetazolamide in SCA6, with assessment using the Ataxia Rating Scale and body stabilometry

1 reference

12 December 2020

Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death

1 reference

12 December 2020

Use of fluoxetine for treatment of Machado-Joseph disease: an open-label study.

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12 December 2020

Recruitment and activation of caspase-8 by the Huntingtin-interacting protein Hip-1 and a novel partner Hippi

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12 December 2020

A cell-based screen for modulators of ataxin-1 phosphorylation

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12 December 2020

Striatal neural grafting improves cortical metabolism in Huntington's disease patients

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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

Recovery from polyglutamine-induced neurodegeneration in conditional SCA1 transgenic mice.

1 reference

12 December 2020

Chemical chaperones reduce aggregate formation and cell death caused by the truncated Machado-Joseph disease gene product with an expanded polyglutamine stretch

1 reference

12 December 2020

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

1 reference

12 December 2020

Human genetic diseases due to codon reiteration: relationship to an evolutionary mechanism

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12 December 2020

Experimental therapeutics in transgenic mouse models of Huntington's disease

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12 December 2020

Trehalose reduces aggregate formation and delays pathology in a transgenic mouse model of oculopharyngeal muscular dystrophy

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12 December 2020

Trehalose alleviates polyglutamine-mediated pathology in a mouse model of Huntington disease

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12 December 2020

10.1007/S12311-008-0026-Z

Identifiers

DOI

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18 August 2017

1 reference

PubMed ID

18418676