Folding Landscape of Mutant Huntingtin Exon1: Diffusible Multimers, Oligomers and Fibrils, and No Detectable Monomer (Q36043574)

11 August 2017

Folding Landscape of Mutant Huntingtin Exon1: Diffusible Multimers, Oligomers and Fibrils, and No Detectable Monomer (English)

1 reference

11 August 2017

Bankanidhi Sahoo

1

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J. Timothy Greenamyre

6

object named as

J Timothy Greenamyre

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11 August 2017

Irene Arduini

2

1 reference

11 August 2017

Kenneth W Drombosky

3

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11 August 2017

Ravindra Kodali

4

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11 August 2017

Laurie H Sanders

5

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11 August 2017

Ronald Wetzel

7

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11 August 2017

language of work or name

English

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publication date

6 June 2016

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11 August 2017

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11 August 2017

11

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11 August 2017

6

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11 August 2017

e0155747

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Creative Commons Attribution 4.0 International

11 August 2017

start time

6 June 2016

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April 2022 Public Data File from Crossref

copyright status

copyrighted

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describes a project that uses

ImageJ

1 reference

11 June 2022

https://www.ebi.ac.uk/europepmc/webservices/rest/PMC4894636/fullTextXML

inferred from PubMed Central ID database lookup

cites work

Huntingtin exon 1 fibrils feature an interdigitated β-hairpin-based polyglutamine core

1 reference

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Delayed emergence of subdiffraction-sized mutant huntingtin fibrils following inclusion body formation

2 October 2017

1 reference

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C-Terminal Threonine Reduces Aβ43 Amyloidogenicity Compared with Aβ42.

2 October 2017

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Huntingtin N-Terminal Monomeric and Multimeric Structures Destabilized by Covalent Modification of Heteroatomic Residues

2 October 2017

1 reference

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Anti-PolyQ Antibodies Recognize a Short PolyQ Stretch in Both Normal and Mutant Huntingtin Exon 1.

2 October 2017

1 reference

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Scalable production in human cells and biochemical characterization of full-length normal and mutant huntingtin

2 October 2017

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Molecular interaction between the chaperone Hsc70 and the N-terminal flank of huntingtin exon 1 modulates aggregation

2 October 2017

1 reference

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The role of protein clearance mechanisms in organismal ageing and age-related diseases.

2 October 2017

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Disruption of the nuclear membrane by perinuclear inclusions of mutant huntingtin causes cell-cycle re-entry and striatal cell death in mouse and cell models of Huntington's disease.

2 October 2017

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Super-resolution fluorescence of huntingtin reveals growth of globular species into short fibers and coexistence of distinct aggregates

2 October 2017

1 reference

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Comparative study of naturally occurring huntingtin fragments in Drosophila points to exon 1 as the most pathogenic species in Huntington's disease

2 October 2017

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Measuring protein dynamics in live cells: protocols and practical considerations for fluorescence fluctuation microscopy

2 October 2017

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Inhibition of mitochondrial protein import by mutant huntingtin

2 October 2017

1 reference

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Soluble forms of polyQ-expanded huntingtin rather than large aggregates cause endoplasmic reticulum stress

2 October 2017

1 reference

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LRRK2 mutations cause mitochondrial DNA damage in iPSC-derived neural cells from Parkinson's disease patients: reversal by gene correction

2 October 2017

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Linear and extended: a common polyglutamine conformation recognized by the three antibodies MW1, 1C2 and 3B5H10

2 October 2017

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Huntington's disease: underlying molecular mechanisms and emerging concepts

2 October 2017

1 reference

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Fragments of HdhQ150 mutant huntingtin form a soluble oligomer pool that declines with aggregate deposition upon aging

2 October 2017

1 reference

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Native mutant huntingtin in human brain: evidence for prevalence of full-length monomer

2 October 2017

1 reference

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Physical chemistry of polyglutamine: intriguing tales of a monotonous sequence

2 October 2017

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Fluorescence correlation spectroscopy: past, present, future

2 October 2017

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Slow amyloid nucleation via α-helix-rich oligomeric intermediates in short polyglutamine-containing huntingtin fragments.

2 October 2017

1 reference

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A soluble α-synuclein construct forms a dynamic tetramer

2 October 2017

1 reference

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A two-step path to inclusion formation of huntingtin peptides revealed by number and brightness analysis

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4894636

Tracking mutant huntingtin aggregation kinetics in cells reveals three major populations that include an invariant oligomer pool.

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4894636

Serines 13 and 16 are critical determinants of full-length human mutant huntingtin induced disease pathogenesis in HD mice

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4894636

Identical oligomeric and fibrillar structures captured from the brains of R6/2 and knock-in mouse models of Huntington's disease

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4894636

Cytoplasmic penetration and persistent infection of mammalian cells by polyglutamine aggregates

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4894636

Atomistic simulations of the effects of polyglutamine chain length and solvent quality on conformational equilibria and spontaneous homodimerization

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4894636

Fluorescence correlation spectroscopy shows that monomeric polyglutamine molecules form collapsed structures in aqueous solutions

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4894636

Analysis of molecular recognition features (MoRFs)

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4894636

Expression and characterization of full-length human huntingtin, an elongated HEAT repeat protein

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4894636

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

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4894636

A cell-based screen for drugs to treat Huntington's disease

2 October 2017

1 reference

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SUMO modification of Huntingtin and Huntington's disease pathology

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4894636

Venezuelan kindreds reveal that genetic and environmental factors modulate Huntington's disease age of onset

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4894636

A linear lattice model for polyglutamine in CAG-expansion diseases

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4894636

The threshold for polyglutamine-expansion protein aggregation and cellular toxicity is dynamic and influenced by aging in Caenorhabditis elegans

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4894636

Intranuclear huntingtin increases the expression of caspase-1 and induces apoptosis

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4894636

Inhibition of huntingtin fibrillogenesis by specific antibodies and small molecules: implications for Huntington's disease therapy

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4894636

Rapid characterization of green fluorescent protein fusion proteins on the molecular and cellular level by fluorescence correlation microscopy

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4894636

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

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4894636

Intranuclear diffusion and hybridization state of oligonucleotides measured by fluorescence correlation spectroscopy in living cells

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4894636

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

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4894636

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

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4894636

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

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4894636

Aggregation behavior of chemically synthesized, full-length huntingtin exon1.

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4894636

N-Terminal acetylation is critical for forming α-helical oligomer of α-synuclein

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4894636

The chaperonin TRiC blocks a huntingtin sequence element that promotes the conformational switch to aggregation

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4894636

Impaired ERAD and ER stress are early and specific events in polyglutamine toxicity

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4894636

Detecting amyloid-beta aggregation with fiber-based fluorescence correlation spectroscopy

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4894636

A critical role for amino-terminal glutamine/asparagine repeats in the formation and propagation of a yeast prion

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4894636

Oxidative damage and metabolic dysfunction in Huntington's disease: selective vulnerability of the basal ganglia.

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4894636

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4894636

Fluorescence Correlation Spectroscopy: A Tool to Study Protein Oligomerization and Aggregation In Vitro and In Vivo.

23 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4894636

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

23 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4894636

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

23 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4894636

Abeta protofibrils possess a stable core structure resistant to hydrogen exchange

23 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4894636

Huntingtin spheroids and protofibrils as precursors in polyglutamine fibrilization

23 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4894636

Polyglutamine aggregation behavior in vitro supports a recruitment mechanism of cytotoxicity

23 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4894636

Accurate calculation of the density of proteins.

23 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4894636

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

23 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4894636

Analysis of gene-specific DNA damage and repair using quantitative polymerase chain reaction

23 September 2018

1 reference

12 December 2020

Kinetics and thermodynamics of amyloid assembly using a high-performance liquid chromatography-based sedimentation assay

1 reference

12 December 2020

Observation of metastable Abeta amyloid protofibrils by atomic force microscopy

1 reference

12 December 2020

Polyglutamine and CBP: fatal attraction?

1 reference

12 December 2020

10.1371/JOURNAL.PONE.0155747

Identifiers

DOI

1 reference

11 August 2017

0 references

1 reference

11 August 2017

PubMed publication ID

27271685

1 reference