Cystamine suppresses polyalanine toxicity in a mouse model of oculopharyngeal muscular dystrophy (Q39697142)

14 September 2017

title

Cystamine suppresses polyalanine toxicity in a mouse model of oculopharyngeal muscular dystrophy (English)

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14 September 2017

Janet E Davies

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14 September 2017

Claudia Rose

series ordinal

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14 September 2017

Sovan Sarkar

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14 September 2017

David C Rubinsztein

series ordinal

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14 September 2017

language of work or name

English

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publication date

1 June 2010

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Science Translational Medicine

14 September 2017

published in

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14 September 2017

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14 September 2017

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14 September 2017

page(s)

34ra40

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Oculopharyngeal muscular dystrophy: potential therapies for an aggregate-associated disorder

14 September 2017

cites work

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https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3000723

Short GCG expansions in the PABP2 gene cause oculopharyngeal muscular dystrophy

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3000723

Mammalian, yeast, bacterial, and chemical chaperones reduce aggregate formation and death in a cell model of oculopharyngeal muscular dystrophy

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3000723

Congo red, doxycycline, and HSP70 overexpression reduce aggregate formation and cell death in cell models of oculopharyngeal muscular dystrophy

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3000723

Doxycycline attenuates and delays toxicity of the oculopharyngeal muscular dystrophy mutation in transgenic mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3000723

Myopathy phenotype in transgenic mice expressing mutated PABPN1 as a model of oculopharyngeal muscular dystrophy.

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3000723

Wild-type PABPN1 is anti-apoptotic and reduces toxicity of the oculopharyngeal muscular dystrophy mutation.

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3000723

Oligomerization of polyalanine expanded PABPN1 facilitates nuclear protein aggregation that is associated with cell death

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3000723

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3000723

Soluble expanded PABPN1 promotes cell death in oculopharyngeal muscular dystrophy

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3000723

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3000723

A cell-based assay for aggregation inhibitors as therapeutics of polyglutamine-repeat disease and validation in Drosophila

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3000723

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3000723

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3000723

Cystamine increases L-cysteine levels in Huntington's disease transgenic mouse brain and in a PC12 model of polyglutamine aggregation

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3000723

Transglutaminases: crosslinking enzymes with pleiotropic functions

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3000723

Mechanism for the inhibition of transglutaminase 2 by cystamine

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3000723

Peptides containing glutamine repeats as substrates for transglutaminase-catalyzed cross-linking: relevance to diseases of the nervous system

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3000723

Transglutaminase cross-links in intranuclear inclusions in Huntington disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3000723

Tau protein is cross-linked by transglutaminase in P301L tau transgenic mice.

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3000723

Brain transglutaminase: in vitro crosslinking of human neurofilament proteins into insoluble polymers

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3000723

Cross-linking of beta-amyloid protein precursor catalyzed by tissue transglutaminase.

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3000723

Cross-linking of a synthetic partial-length (1-28) peptide of the Alzheimer beta/A4 amyloid protein by transglutaminase.

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3000723

Two isoforms of tissue transglutaminase mediate opposing cellular fates

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3000723

Tissue transglutaminase differentially modulates apoptosis in a stimuli-dependent manner

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3000723

Tissue transglutaminase serves as an inhibitor of apoptosis by cross-linking caspase 3 in thapsigargin-treated cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3000723

Tissue transglutaminase during mouse central nervous system development: lack of alternative RNA processing and implications for its role(s) in murine models of neurotrauma and neurodegeneration

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3000723

Site-directed mutagenesis of human tissue transglutaminase: Cys-277 is essential for transglutaminase activity but not for GTPase activity.

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3000723

The protective effects of cystamine in the R6/2 Huntington's disease mouse involve mechanisms other than the inhibition of tissue transglutaminase

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3000723

Cystamine treatment is neuroprotective in the YAC128 mouse model of Huntington disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3000723

Cystamine inhibits transglutaminase and caspase-3 cleavage in glutamate-exposed astroglial cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3000723

Identification of glutamine and lysine residues in Alzheimer amyloid beta A4 peptide responsible for transglutaminase-catalysed homopolymerization and cross-linking to alpha 2M receptor.

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3000723

Movement of Bax from the Cytosol to Mitochondria during Apoptosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3000723

10.1126/SCITRANSLMED.3000723

21 January 2018

Identifiers

DOI

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14 September 2017

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