Protein misfolding detected early in pathogenesis of transgenic mouse model of Huntington disease using amyloid seeding assay (Q35880053)

10 August 2017

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

1 reference

10 August 2017

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Sharad Gupta

1

1 reference

10 August 2017

Shy'Ann Jie

series ordinal

2

1 reference

10 August 2017

David W Colby

series ordinal

3

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10 August 2017

language of work or name

English

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publication date

20 December 2011

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10 August 2017

8

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Journal of Biological Chemistry

inferred from page(s)

published in

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10 August 2017

287

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10 August 2017

13

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10 August 2017

9982-9989

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Huntington's disease: can mice lead the way to treatment?

10 August 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3323021

Ultrasensitive human prion detection in cerebrospinal fluid by real-time quaking-induced conversion.

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3323021

A kinetic aggregation assay allowing selective and sensitive amyloid-β quantification in cells and tissues

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3323021

Rapid end-point quantitation of prion seeding activity with sensitivity comparable to bioassays

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3323021

Brief ampakine treatments slow the progression of Huntington's disease phenotypes in R6/2 mice

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3323021

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

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3323021

Protease-sensitive synthetic prions

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3323021

A causative link between the structure of aberrant protein oligomers and their 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=3323021

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

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3323021

Neuroanatomic profile of polyglutamine immunoreactivity in Huntington disease brains.

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3323021

Tiagabine is neuroprotective in the N171-82Q and R6/2 mouse models of Huntington's disease

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3323021

Prion detection by an amyloid seeding assay.

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3323021

Neuronal intranuclear and neuropil inclusions for pathological assessment of Huntington's disease

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3323021

RNA interference improves motor and neuropathological abnormalities in a Huntington's disease mouse model

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3323021

Potent inhibition of huntingtin aggregation and cytotoxicity by a disulfide bond-free single-domain intracellular antibody

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3323021

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

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3323021

Time course of early motor and neuropathological anomalies in a knock-in mouse model of Huntington's disease with 140 CAG repeats

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3323021

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

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3323021

Neuroprotective effects of creatine in a transgenic mouse model of Huntington's disease.

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3323021

Long glutamine tracts cause nuclear localization of a novel form of huntingtin in medium spiny striatal neurons in HdhQ92 and HdhQ111 knock-in mice

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3323021

Ultrastructural localization and progressive formation of neuropil aggregates in Huntington's disease transgenic mice

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3323021

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

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3323021

Neuronal distribution of intranuclear inclusions in Huntington's disease with adult onset.

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3323021

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

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3323021

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

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3323021

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

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3323021

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

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3323021

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

11 July 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M111.305417

Aggregated polyglutamine peptides delivered to nuclei are toxic to mammalian cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M111.305417

Beneficial effects of rolipram in the R6/2 mouse model of Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M111.305417

Intranuclear inclusions and neuritic aggregates in transgenic mice expressing a mutant N-terminal fragment of huntingtin

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M111.305417

Creatine increase survival and delays motor symptoms in a transgenic animal model of Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M111.305417

Neuroprotective effects of a novel kynurenic acid analogue in a transgenic mouse model of Huntington's disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M111.305417

A YAC mouse model for Huntington's disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M111.305417

Selective striatal neuronal loss in a YAC128 mouse model of Huntington disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M111.305417

Cognitive dysfunction precedes neuropathology and motor abnormalities in the YAC128 mouse model of Huntington's disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M111.305417

Phenotypic abnormalities in the YAC128 mouse model of Huntington disease are penetrant on multiple genetic backgrounds and modulated by strain

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M111.305417

Biochemical, ultrastructural, and reversibility studies on huntingtin filaments isolated from mouse and human brain.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M111.305417

Polyglutamine aggregation behavior in vitro supports a recruitment mechanism of cytotoxicity

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M111.305417

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M111.305417

Distinct behavioral and neuropathological abnormalities in transgenic mouse models of HD and DRPLA.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M111.305417

Selective degeneration and nuclear localization of mutant huntingtin in the YAC128 mouse model of Huntington disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M111.305417

Stochastic kinetics of intracellular huntingtin aggregate formation.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M111.305417

Therapeutic effects of cystamine in a murine model 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=3323021

Thioflavin-T for Amyloid Detection

24 September 2018

1 reference

PubMed

https://pubmed.ncbi.nlm.nih.gov/22187438

12 December 2020

inferred from PubMed ID database lookup

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

1 reference

PubMed

https://pubmed.ncbi.nlm.nih.gov/22187438

12 December 2020

inferred from PubMed ID database lookup

Interference by huntingtin and atrophin-1 with cbp-mediated transcription leading to cellular toxicity

1 reference

PubMed

https://pubmed.ncbi.nlm.nih.gov/22187438

12 December 2020

inferred from PubMed ID database lookup

Identifiers

DOI

10.1074/JBC.M111.305417

1 reference

10 August 2017

1 reference

10 August 2017

PubMed publication ID

22187438

1 reference