Mutant huntingtin fragments form oligomers in a polyglutamine length-dependent manner in vitro and in vivo (Q30494325)

19 September 2017

Mutant huntingtin fragments form oligomers in a polyglutamine length-dependent manner in vitro and in vivo (English)

1 reference

19 September 2017

Justin Legleiter

1

1 reference

19 September 2017

Emily Mitchell

2

1 reference

19 September 2017

Gregor P Lotz

3

1 reference

19 September 2017

Ellen Sapp

4

1 reference

19 September 2017

Cheping Ng

5

1 reference

19 September 2017

Marian DiFiglia

6

1 reference

19 September 2017

Leslie M Thompson

7

1 reference

19 September 2017

Paul J Muchowski

8

1 reference

19 September 2017

language of work or name

English

0 references

publication date

10 March 2010

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Journal of Biological Chemistry

19 September 2017

14

1 reference

based on heuristic

inferred from page(s)

published in

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19 September 2017

285

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19 September 2017

14777-14790

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19 September 2017

19

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Intrabody gene therapy ameliorates motor, cognitive, and neuropathological symptoms in multiple mouse models of Huntington's disease

19 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2863238

Secondary Structure of Huntingtin Amino-Terminal Region

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2863238

Monoclonal antibodies recognize distinct conformational epitopes formed by polyglutamine in a mutant huntingtin fragment

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2863238

Distinct conformations of in vitro and in vivo amyloids of huntingtin-exon1 show different cytotoxicity

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2863238

Mutant huntingtin N-terminal fragments of specific size mediate aggregation and toxicity in neuronal cells

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2863238

Soluble polyglutamine oligomers formed prior to inclusion body formation are cytotoxic

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2863238

Endocytosis machinery is involved in aggregation of proteins with expanded polyglutamine domains

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2863238

The interplay between PolyQ and protein context delays aggregation by forming a reservoir of protofibrils

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2863238

Flanking sequences profoundly alter polyglutamine toxicity in yeast

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2863238

A network of protein interactions determines polyglutamine toxicity

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2863238

Expansion of amino acid homo-sequences in proteins: insights into the role of amino acid homo-polymers and of the protein context in aggregation

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2863238

Extended polyglutamine tracts cause aggregation and structural perturbation of an adjacent beta barrel protein

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2863238

polyglutamine aggregation nucleation: thermodynamics of a highly unfavorable protein folding reaction

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2863238

Alterations in N-methyl-D-aspartate receptor sensitivity and magnesium blockade occur early in development in the R6/2 mouse model of Huntington's disease

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2863238

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

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2863238

SUMO modification of Huntingtin and Huntington's disease pathology

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2863238

Huntingtin bodies sequester vesicle-associated proteins by a polyproline-dependent interaction.

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2863238

Polyglutamines placed into context

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2863238

Mutational analysis of the structural organization of polyglutamine aggregates

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2863238

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

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2863238

Requirement of an intact microtubule cytoskeleton for aggregation and inclusion body formation by a mutant huntingtin fragment.

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2863238

New anti-huntingtin monoclonal antibodies: implications for huntingtin conformation and its binding proteins

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2863238

Huntington's disease: the challenge for cell biologists

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2863238

Hsp70 and hsp40 chaperones can inhibit self-assembly of polyglutamine proteins into amyloid-like fibrils

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2863238

Glutamine repeats and neurodegeneration

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2863238

Insoluble detergent-resistant aggregates form between pathological and nonpathological lengths of polyglutamine in mammalian cells

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2863238

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

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2863238

In situ atomic force microscopy study of Alzheimer's beta-amyloid peptide on different substrates: new insights into mechanism of beta-sheet formation

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2863238

Nuclear and neuropil aggregates in Huntington's disease: relationship to neuropathology.

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2863238

A cellular model that recapitulates major pathogenic steps of Huntington's disease

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2863238

The influence of huntingtin protein size on nuclear localization and cellular toxicity.

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2863238

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

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2863238

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

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2863238

CAG repeat number governs the development rate of pathology in Huntington's disease

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2863238

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

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2863238

A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes.

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2863238

Relationship between trinucleotide repeat expansion and phenotypic variation in Huntington's disease

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2863238

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

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2863238

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

20 June 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M109.093708

Amyloid-like features of polyglutamine aggregates and their assembly kinetics

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M109.093708

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M109.093708

Huntingtin spheroids and protofibrils as precursors in polyglutamine fibrilization

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M109.093708

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M109.093708

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M109.093708

Polyglutamine aggregation behavior in vitro supports a recruitment mechanism of cytotoxicity

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M109.093708

In situ AFM studies of astrocyte-secreted apolipoprotein E- and J-containing lipoproteins.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M109.093708

Oligoproline effects on polyglutamine conformation and aggregation

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M109.093708

Polyglutamine disruption of the huntingtin exon 1 N terminus triggers a complex aggregation mechanism

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M109.093708

Polyglutamine dances the conformational cha-cha-cha

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M109.093708

Flanking polyproline sequences inhibit beta-sheet structure in polyglutamine segments by inducing PPII-like helix structure

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M109.093708

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M109.093708

Detection of polyglutamine protein oligomers in cells by fluorescence correlation spectroscopy

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M109.093708

Sodium dodecyl sulfate-insoluble oligomers are involved in polyglutamine degeneration.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M109.093708

Increased sensitivity to N-methyl-D-aspartate receptor-mediated excitotoxicity in a mouse model of Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M109.093708

In-situ atomic force microscopy study of β-amyloid fibrillization

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M109.093708

Sensitive biochemical aggregate detection reveals aggregation onset before symptom development in cellular and murine models of Huntington's disease

21 January 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2863238

A toxic monomeric conformer of the polyglutamine protein

26 November 2018

1 reference

PubMed

https://pubmed.ncbi.nlm.nih.gov/20220138

inferred from PubMed ID database lookup

12 December 2020

based on heuristic

Identifiers

DOI

10.1074/JBC.M109.093708

1 reference

19 September 2017

1 reference

19 September 2017

1 reference