(Q33788913)

29 July 2017

title

Monomeric, oligomeric and polymeric proteins in huntington disease and other diseases of polyglutamine expansion (English)

1 reference

29 July 2017

0 references

1

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:24961702%20AND%20SRC:MED&resulttype=core&format=json

9 June 2020

Philippe Djian

series ordinal

2

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:24961702%20AND%20SRC:MED&resulttype=core&format=json

9 June 2020

author name string

Guylaine Hoffner

series ordinal

1

1 reference

29 July 2017

Philippe Djian

series ordinal

2

1 reference

29 July 2017

publication date

3 March 2014

1 reference

29 July 2017

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:24961702%20AND%20SRC:MED&resulttype=core&format=json

9 June 2020

volume

4

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:24961702%20AND%20SRC:MED&resulttype=core&format=json

9 June 2020

issue

1

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:24961702%20AND%20SRC:MED&resulttype=core&format=json

9 June 2020

page(s)

91-122

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:24961702%20AND%20SRC:MED&resulttype=core&format=json

Creative Commons Attribution 4.0 International

9 June 2020

copyright license

start time

3 March 2014

1 reference

April 2022 Public Data File from Crossref

copyright status

copyrighted

0 references

cites work

Polyglutamine domain flexibility mediates the proximity between flanking sequences in huntingtin

1 reference

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

4 July 2018

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The interaction of polyglutamine peptides with lipid membranes is regulated by flanking sequences associated with huntingtin

4 July 2018

1 reference

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Aberrant splicing of HTT generates the pathogenic exon 1 protein in Huntington disease

4 July 2018

1 reference

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

4 July 2018

1 reference

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Structural features and domain organization of huntingtin fibrils

4 July 2018

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Fibrillar α-synuclein and huntingtin exon 1 assemblies are toxic to the cells

4 July 2018

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Neuronal properties, in vivo effects, and pathology of a Huntington's disease patient-derived induced pluripotent stem cells

4 July 2018

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Astrocytes generated from patient induced pluripotent stem cells recapitulate features of Huntington's disease patient cells

4 July 2018

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Structural features and cytotoxicity of amyloid oligomers: implications in Alzheimer's disease and other diseases with amyloid deposits

4 July 2018

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Amyloid-like fibril formation by polyQ proteins: a critical balance between the polyQ length and the constraints imposed by the host protein

4 July 2018

1 reference

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Identification of novel potentially toxic oligomers formed in vitro from mammalian-derived expanded huntingtin exon-1 protein

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

The first reported generation of several induced pluripotent stem cell lines from homozygous and heterozygous Huntington's disease patients demonstrates mutation related enhanced lysosomal activity

4 July 2018

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Disease-Associated Polyglutamine Stretches in Monomeric Huntingtin Adopt a Compact Structure

4 July 2018

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Induced pluripotent stem cell lines from Huntington's disease mice undergo neuronal differentiation while showing alterations in the lysosomal pathway

4 July 2018

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Transglutaminase in epidermis and neurological disease or what makes a good cross-linking substrate.

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Protein misfolding detected early in pathogenesis of transgenic mouse model of Huntington disease using amyloid seeding assay

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Inhibiting the nucleation of amyloid structure in a huntingtin fragment by targeting α-helix-rich oligomeric intermediates

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

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

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Excitation-induced ataxin-3 aggregation in neurons from patients with Machado-Joseph disease

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Identifying polyglutamine protein species in situ that best predict neurodegeneration

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

A major role for side-chain polyglutamine hydrogen bonding in irreversible ataxin-3 aggregation

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

The aggregation-enhancing huntingtin N-terminus is helical in amyloid fibrils

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Critical nucleus size for disease-related polyglutamine aggregation is repeat-length dependent

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Perturbation with intrabodies reveals that calpain cleavage is required for degradation of huntingtin exon 1

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

A compact beta model of huntingtin toxicity

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Essential role of coiled coils for aggregation and activity of Q/N-rich prions and PolyQ proteins

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Biology of amyloid: structure, function, and regulation

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Reprogramming Huntington monkey skin cells into pluripotent stem cells

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

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

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Polyglutamine induced misfolding of huntingtin exon1 is modulated by the flanking sequences

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Neural mechanisms of ageing and cognitive decline

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

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

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Proteolysis of mutant huntingtin produces an exon 1 fragment that accumulates as an aggregated protein in neuronal nuclei in Huntington disease

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

A cellular perspective on conformational disease: the role of genetic background and proteostasis networks

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Protein folding: sticky N17 speeds huntingtin pile-up

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

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

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Mechanism of cis-inhibition of polyQ fibrillation by polyP: PPII oligomers and the hydrophobic effect

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

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

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Secondary Structure of Huntingtin Amino-Terminal Region

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Single homopolypeptide chains collapse into mechanically rigid conformations

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

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

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

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

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Accumulation of N-terminal mutant huntingtin in mouse and monkey models implicated as a pathogenic mechanism in Huntington's disease.

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Cerebral cortex and the clinical expression of Huntington's disease: complexity and heterogeneity

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Soluble polyglutamine oligomers formed prior to inclusion body formation are cytotoxic

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

In-cell aggregation of a polyglutamine-containing chimera is a multistep process initiated by the flanking sequence

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Huntingtin has a membrane association signal that can modulate huntingtin aggregation, nuclear entry and toxicity

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Prevention of amyloid-like aggregation as a driving force of protein evolution.

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

On the structural definition of amyloid fibrils and other polypeptide aggregates

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

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

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Kinetics and thermodynamics of amyloid fibril assembly

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Normal-repeat-length polyglutamine peptides accelerate aggregation nucleation and cytotoxicity of expanded polyglutamine proteins

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Critical role of the proline-rich region in Huntingtin for aggregation and cytotoxicity in yeast.

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Flanking sequences profoundly alter polyglutamine toxicity in yeast

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Evidence that Perutz's double-beta-stranded subunit structure for beta-amyloids also applies to their channel-forming structures in membranes

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Absence of behavioral abnormalities and neurodegeneration in vivo despite widespread neuronal huntingtin inclusions

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Formation of morphologically similar globular aggregates from diverse aggregation-prone proteins in mammalian cells

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

4 July 2018

Amyloid peptide channels

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

A structure-based analysis of huntingtin mutant polyglutamine aggregation and toxicity: evidence for a compact beta-sheet structure.

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

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

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Cellular toxicity of polyglutamine expansion proteins: mechanism of transcription factor deactivation

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

SUMO modification of Huntingtin and Huntington's disease pathology

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Huntingtin forms toxic NH2-terminal fragment complexes that are promoted by the age-dependent decrease in proteasome activity.

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

A camelid antibody fragment inhibits the formation of amyloid fibrils by human lysozyme

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Protofibrils, pores, fibrils, and neurodegeneration: separating the responsible protein aggregates from the innocent bystanders

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Common structure of soluble amyloid oligomers implies common mechanism of pathogenesis

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Parkin facilitates the elimination of expanded polyglutamine proteins and leads to preservation of proteasome function.

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Oligomeric and polymeric aggregates formed by proteins containing expanded polyglutamine

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Mutational analysis of the structural organization of polyglutamine aggregates

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Caspase cleavage of mutant huntingtin precedes neurodegeneration in Huntington's disease.

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

A linear lattice model for polyglutamine in CAG-expansion diseases

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Proteases acting on mutant huntingtin generate cleaved products that differentially build up cytoplasmic and nuclear inclusions

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

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

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Calpain activation in Huntington's disease.

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Sp1 and TAFII130 transcriptional activity disrupted in early Huntington's disease

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Amyloid fibers are water-filled nanotubes

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

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.

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

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

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

SCA17, a novel autosomal dominant cerebellar ataxia caused by an expanded polyglutamine in TATA-binding protein

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Caretaker or undertaker? The role of the proteasome in aging

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Identities of sequestered proteins in aggregates from cells with induced polyglutamine expression

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Formic acid dissolves aggregates of an N-terminal huntingtin fragment containing an expanded polyglutamine tract: applying to quantification of protein components of the aggregates

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

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

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Poly-L-glutamine forms cation channels: relevance to the pathogenesis of the polyglutamine diseases

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

The Huntington's disease protein interacts with p53 and CREB-binding protein and represses transcription

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Abundant expression and cytoplasmic aggregations of [alpha]1A voltage-dependent calcium channel protein associated with neurodegeneration in spinocerebellar ataxia type 6

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Huntington's disease intranuclear inclusions contain truncated, ubiquitinated huntingtin protein

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

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

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Hereditary dentatorubral-pallidoluysian atrophy: detection of widespread ubiquitinated neuronal and glial intranuclear inclusions in the brain

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

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

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Transglutaminase action imitates Huntington's disease: selective polymerization of Huntingtin containing expanded polyglutamine.

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Truncated N-terminal fragments of huntingtin with expanded glutamine repeats form nuclear and cytoplasmic aggregates in cell culture.

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Length of huntingtin and its polyglutamine tract influences localization and frequency of intracellular aggregates

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Common core structure of amyloid fibrils by synchrotron X-ray diffraction.

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Ataxin-1 with an expanded glutamine tract alters nuclear matrix-associated structures

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

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

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Cloning of the SCA7 gene reveals a highly unstable CAG repeat expansion

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Random coil conformation for extended polyglutamine stretches in aqueous soluble monomeric peptides.

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

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

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

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

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

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

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

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

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Cloning of the gene for spinocerebellar ataxia 2 reveals a locus with high sensitivity to expanded CAG/glutamine repeats

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Identification of the spinocerebellar ataxia type 2 gene using a direct identification of repeat expansion and cloning technique, DIRECT.

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Moderate expansion of a normally biallelic trinucleotide repeat in spinocerebellar ataxia type 2.

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Cleavage of huntingtin by apopain, a proapoptotic cysteine protease, is modulated by the polyglutamine tract

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Phenotypic characterization of individuals with 30-40 CAG repeats in the Huntington disease (HD) gene reveals HD cases with 36 repeats and apparently normal elderly individuals with 36-39 repeats

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Codon repeats in genes associated with human diseases: fewer repeats in the genes of nonhuman primates and nucleotide substitutions concentrated at the sites of reiteration

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

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

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Expansion of an unstable trinucleotide CAG repeat in spinocerebellar ataxia type 1.

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Glutamine repeats as polar zippers: their possible role in inherited neurodegenerative diseases

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Unstable expansion of CAG repeat in hereditary dentatorubral-pallidoluysian atrophy (DRPLA)

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

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

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Cellular localization of the Huntington's disease protein and discrimination of the normal and mutated form

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Incorporation of glutamine repeats makes protein oligomerize: implications for neurodegenerative diseases

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Expression of the Huntington's disease (IT15) protein product in HD patients

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

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

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Neuropathological classification of Huntington's disease

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Androgen receptor gene mutations in X-linked spinal and bulbar muscular atrophy

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

Different types of repeat expansion in the TATA-binding protein gene are associated with a new form of inherited ataxia

4 July 2018

1 reference

https://api.crossref.org/works/10.3390%2FBRAINSCI4010091

Purification of neuronal inclusions of patients with Huntington's disease reveals a broad range of N-terminal fragments of expanded huntingtin and insoluble polymers

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FBRAINSCI4010091

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

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FBRAINSCI4010091

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

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FBRAINSCI4010091

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

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FBRAINSCI4010091

Neuronal intranuclear inclusions in spinocerebellar ataxia type 2: triple-labeling immunofluorescent study

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FBRAINSCI4010091

CAG repeat expansion in the TATA box-binding protein gene causes autosomal dominant cerebellar ataxia.

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FBRAINSCI4010091

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/10.3390%2FBRAINSCI4010091

Amyloid-like features of polyglutamine aggregates and their assembly kinetics

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FBRAINSCI4010091

Amyloid formation by mutant huntingtin: threshold, progressivity and recruitment of normal polyglutamine proteins

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FBRAINSCI4010091

Amyloid-like inclusions in Huntington's disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FBRAINSCI4010091

Nuclear inclusions in spinocerebellar ataxia type 1.

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FBRAINSCI4010091

Differential morphology and composition of inclusions in the R6/2 mouse and PC12 cell models of Huntington's disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FBRAINSCI4010091

Structural characterization of polyglutamine fibrils by solid-state NMR spectroscopy

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FBRAINSCI4010091

Structure of inclusions of Huntington's disease brain revealed by synchrotron infrared microspectroscopy: polymorphism and relevance to cytotoxicity

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FBRAINSCI4010091

A one-hit model of cell death in inherited neuronal degenerations

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FBRAINSCI4010091

Cause of neural death in neurodegenerative diseases attributable to expansion of glutamine repeats

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FBRAINSCI4010091

Cross-seeding fibrillation of Q/N-rich proteins offers new pathomechanism of polyglutamine diseases

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FBRAINSCI4010091

Aging causes distinct characteristics of polyglutamine amyloids in vivo

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FBRAINSCI4010091

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

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FBRAINSCI4010091

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

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FBRAINSCI4010091

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

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FBRAINSCI4010091

The first 17 amino acids of Huntingtin modulate its sub-cellular localization, aggregation and effects on calcium homeostasis.

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FBRAINSCI4010091

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/10.3390%2FBRAINSCI4010091

Mutational bias provides a model for the evolution of Huntington's disease and predicts a general increase in disease prevalence.

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FBRAINSCI4010091

Oligoproline effects on polyglutamine conformation and aggregation

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FBRAINSCI4010091

Induced pluripotent stem cells from patients with Huntington's disease show CAG-repeat-expansion-associated phenotypes

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FBRAINSCI4010091

Cleavage at the caspase-6 site is required for neuronal dysfunction and degeneration due to mutant huntingtin

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FBRAINSCI4010091

Aggregated polyglutamine peptides delivered to nuclei are toxic to mammalian cells

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FBRAINSCI4010091

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/10.3390%2FBRAINSCI4010091

Paradoxical delay in the onset of disease caused by super-long CAG repeat expansions in R6/2 mice.

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FBRAINSCI4010091

Huntingtin spheroids and protofibrils as precursors in polyglutamine fibrilization

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FBRAINSCI4010091

Soluble androgen receptor oligomers underlie pathology in a mouse model of spinobulbar muscular atrophy

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FBRAINSCI4010091

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

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FBRAINSCI4010091

Green tea (-)-epigallocatechin-gallate modulates early events in huntingtin misfolding and reduces toxicity in Huntington's disease models

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FBRAINSCI4010091

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

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FBRAINSCI4010091

Folding of oligoglutamines: a theoretical approach based upon thermodynamics and molecular mechanics.

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FBRAINSCI4010091

Solution structure of polyglutamine tracts in GST-polyglutamine fusion proteins

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FBRAINSCI4010091

Characterizing the conformational ensemble of monomeric polyglutamine.

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FBRAINSCI4010091

Mutant huntingtin promotes the fibrillogenesis of wild-type huntingtin: a potential mechanism for loss of huntingtin function in Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FBRAINSCI4010091

Pathogenic and Non-pathogenic Polyglutamine Tracts Have Similar Structural Properties: Towards a Length-dependent Toxicity Gradient

21 January 2018

1 reference

https://api.crossref.org/works/10.3390%2FBRAINSCI4010091

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=4066239

Metabolic network abnormalities in early Huntington's disease: an [(18)F]FDG PET study.

30 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4066239

X-ray diffraction studies on amyloid filaments

30 October 2018

1 reference

12 December 2020

1 reference

12 December 2020

The role of protein composition in specifying nuclear inclusion formation in polyglutamine disease

1 reference

12 December 2020

Polyglutamine homopolymers having 8-45 residues form slablike beta-crystallite assemblies

1 reference

12 December 2020

Characterization of the Amyloid Fibril as a Cross- Protein

1 reference

12 December 2020

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

1 reference

12 December 2020

A toxic monomeric conformer of the polyglutamine protein

1 reference

12 December 2020

Identifiers

DOI

10.3390/BRAINSCI4010091

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:24961702%20AND%20SRC:MED&resulttype=core&format=json

9 June 2020

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:24961702%20AND%20SRC:MED&resulttype=core&format=json

9 June 2020

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:24961702%20AND%20SRC:MED&resulttype=core&format=json