(Q45042684)

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:15345714%20AND%20SRC:MED&resulttype=core&format=json

1 January 2020

title

Polyglutamine expansion in ataxin-3 does not affect protein stability: implications for misfolding and disease (English)

1 reference

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1 January 2020

1

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1 January 2020

Andrew M Ellisdon

series ordinal

2

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1 January 2020

4

Stephen P Bottomley

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1 January 2020

author name string

Lisa D Cabrita

series ordinal

3

1 reference

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1 January 2020

language of work or name

English

0 references

publication date

1 September 2004

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:15345714%20AND%20SRC:MED&resulttype=core&format=json

Journal of Biological Chemistry

1 January 2020

published in

1 reference

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1 January 2020

volume

279

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1 January 2020

issue

46

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1 January 2020

page(s)

47643-47651

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

1 January 2020

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Glutamine repeats and neurodegeneration

21 January 2018

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Ubiquitin-mediated sequestration of normal cellular proteins into polyglutamine aggregates

21 January 2018

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Ataxin-3 interactions with rad23 and valosin-containing protein and its associations with ubiquitin chains and the proteasome are consistent with a role in ubiquitin-mediated proteolysis

21 January 2018

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The polyglutamine neurodegenerative protein ataxin-3 binds polyubiquitylated proteins and has ubiquitin protease activity

21 January 2018

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CAG expansions in a novel gene for Machado-Joseph disease at chromosome 14q32.1

21 January 2018

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

21 January 2018

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Spinocerebellar ataxia type 7 (SCA7): a neurodegenerative disorder with neuronal intranuclear inclusions.

21 January 2018

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

21 January 2018

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Protein aggregation and aggregate toxicity: new insights into protein folding, misfolding diseases and biological evolution

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Promiscuous beta-strand interactions and the conformational diseases.

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The role of protein misfolding in the pathogenesis of human diseases.

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Amyloid-fibril formation. Proposed mechanisms and relevance to conformational disease

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Principles of protein folding, misfolding and aggregation

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21 January 2018

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

21 January 2018

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Amyloid-like inclusions in Huntington's disease.

21 January 2018

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An expanded glutamine repeat destabilizes native ataxin-3 structure and mediates formation of parallel beta -fibrils.

21 January 2018

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Amyloid-like features of polyglutamine aggregates and their assembly kinetics

21 January 2018

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Huntingtin spheroids and protofibrils as precursors in polyglutamine fibrilization

21 January 2018

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Intra- and intermolecular beta-pleated sheet formation in glutamine-repeat inserted myoglobin as a model for polyglutamine diseases

21 January 2018

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Polyglutamine aggregation behavior in vitro supports a recruitment mechanism of cytotoxicity

21 January 2018

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Destabilization of a non-pathological variant of ataxin-3 results in fibrillogenesis via a partially folded intermediate: a model for misfolding in polyglutamine disease.

21 January 2018

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Temperature-dependent, irreversible formation of amyloid fibrils by a soluble human ataxin-3 carrying a moderately expanded polyglutamine stretch (Q36).

21 January 2018

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

21 January 2018

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Structural instability and fibrillar aggregation of non-expanded human ataxin-3 revealed under high pressure and temperature

21 January 2018

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The alternative conformations of amyloidogenic proteins and their multi-step assembly pathways

21 January 2018

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Machado-Joseph disease gene products carrying different carboxyl termini

21 January 2018

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Estimation of globular protein secondary structure from circular dichroism.

21 January 2018

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DICHROWEB: an interactive website for the analysis of protein secondary structure from circular dichroism spectra.

21 January 2018

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A linear lattice model for polyglutamine in CAG-expansion diseases

21 January 2018

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

21 January 2018

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Expanded polyglutamine protein forms nuclear inclusions and causes neural degeneration in Drosophila.

21 January 2018

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Polyglutamine expansion, protein aggregation, proteasome activity, and neural survival.

21 January 2018

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Domain architecture of the polyglutamine protein ataxin-3: a globular domain followed by a flexible tail

21 January 2018

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The structural basis of protein folding and its links with human disease

21 January 2018

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Familial mutations and the thermodynamic stability of the recombinant human prion protein.

21 January 2018

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Influence of amino acid substitutions related to inherited human prion diseases on the thermodynamic stability of the cellular prion protein.

21 January 2018

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Aggregation of the Acylphosphatase from Sulfolobus solfataricus: the folded and partially unfolded states can both be precursors for amyloid formation.

21 January 2018

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The effect of net charge on the solubility, activity, and stability of ribonuclease Sa

21 January 2018

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The role of protein stability, solubility, and net charge in amyloid fibril formation

21 January 2018

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Polar zippers: their role in human disease

21 January 2018

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Mutational analysis of the structural organization of polyglutamine aggregates

21 January 2018

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The regions of the sequence most exposed to the solvent within the amyloidogenic state of a protein initiate the aggregation process.

21 January 2018

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Huntington's disease age-of-onset linked to polyglutamine aggregation nucleation

21 January 2018

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Amino acid sequences flanking polyglutamine stretches influence their potential for aggregate formation

21 January 2018

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

21 January 2018

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Inhibiting caspase cleavage of huntingtin reduces toxicity and aggregate formation in neuronal and nonneuronal cells

21 January 2018

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Kennedy's disease: caspase cleavage of the androgen receptor is a crucial event in cytotoxicity

21 January 2018

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Caspase 3-cleaved N-terminal fragments of wild-type and mutant huntingtin are present in normal and Huntington's disease brains, associate with membranes, and undergo calpain-dependent proteolysis.

21 January 2018

1 reference

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Structural and functional analysis of the Josephin domain of the polyglutamine protein ataxin-3

21 January 2018

1 reference

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21 January 2018

Identifiers

DOI

10.1074/JBC.M405799200

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:15345714%20AND%20SRC:MED&resulttype=core&format=json

1 January 2020

1 reference