Huntingtin cleavage product A forms in neurons and is reduced by gamma-secretase inhibitors (Q41881745)

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:21156064%20AND%20SRC:MED&resulttype=core&format=json

18 January 2020

title

Huntingtin cleavage product A forms in neurons and is reduced by gamma-secretase inhibitors (English)

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18 January 2020

17

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18 January 2020

author name string

Kimberly B Kegel

1

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18 January 2020

Ellen Sapp

2

1 reference

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18 January 2020

Jonathan Alexander

3

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18 January 2020

Patrick Reeves

4

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18 January 2020

Dorothee Bleckmann

5

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18 January 2020

Linsday Sobin

6

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18 January 2020

Nicholas Masso

7

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18 January 2020

Antonio Valencia

8

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18 January 2020

Hyunkyung Jeong

9

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18 January 2020

Dimitri Krainc

10

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18 January 2020

James Palacino

11

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18 January 2020

Daniel Curtis

12

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18 January 2020

Rainer Kuhn

13

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18 January 2020

Claudia Betschart

14

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18 January 2020

Miguel Sena-Esteves

15

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18 January 2020

Neil Aronin

16

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18 January 2020

Marian Difiglia

18

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18 January 2020

publication date

14 December 2010

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Molecular Neurodegeneration

18 January 2020

published in

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18 January 2020

volume

5

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18 January 2020

page(s)

58

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https://scigraph.springernature.com/pub.10.1186/1750-1326-5-58

18 January 2020

exact match

0 references

cites work

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

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Differential activities of the ubiquitin-proteasome system in neurons versus glia may account for the preferential accumulation of misfolded proteins in neurons

5 June 2018

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5 June 2018

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Full-length human mutant huntingtin with a stable polyglutamine repeat can elicit progressive and selective neuropathogenesis in BACHD mice

5 June 2018

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Direct and potent regulation of gamma-secretase by its lipid microenvironment

5 June 2018

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5 June 2018

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Therapeutic silencing of mutant huntingtin with siRNA attenuates striatal and cortical neuropathology and behavioral deficits

5 June 2018

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

5 June 2018

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Characterization of huntingtin pathologic fragments in human Huntington disease, transgenic mice, and cell models.

5 June 2018

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Huntingtin cleavage induced by thrombin in vitro

5 June 2018

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Lysosomal proteases are involved in generation of N-terminal huntingtin fragments

5 June 2018

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Interaction of huntingtin fragments with brain membranes--clues to early dysfunction in Huntington's disease

5 June 2018

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gamma-Secretase substrate selectivity can be modulated directly via interaction with a nucleotide-binding site

5 June 2018

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Contribution of nuclear and extranuclear polyQ to neurological phenotypes in mouse models of Huntington's disease

5 June 2018

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Huntingtin associates with acidic phospholipids at the plasma membrane

5 June 2018

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Structure-based design, synthesis, and memapsin 2 (BACE) inhibitory activity of carbocyclic and heterocyclic peptidomimetics

5 June 2018

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

5 June 2018

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Association of active gamma-secretase complex with lipid rafts.

5 June 2018

1 reference

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Gamma-secretase exists on the plasma membrane as an intact complex that accepts substrates and effects intramembrane cleavage

5 June 2018

1 reference

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Chronic treatment with the gamma-secretase inhibitor LY-411,575 inhibits beta-amyloid peptide production and alters lymphopoiesis and intestinal cell differentiation

5 June 2018

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

5 June 2018

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5 June 2018

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5 June 2018

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5 June 2018

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

5 June 2018

1 reference

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

5 June 2018

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The channel hypothesis of Huntington's disease

5 June 2018

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Changes in cortical and striatal neurons predict behavioral and electrophysiological abnormalities in a transgenic murine model of Huntington's disease

5 June 2018

1 reference

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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.

5 June 2018

1 reference

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Mutant protein in Huntington disease is resistant to proteolysis in affected brain

5 June 2018

1 reference

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Photoactivated gamma-secretase inhibitors directed to the active site covalently label presenilin 1

5 June 2018

1 reference

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Self-inactivating lentiviral vectors with enhanced transgene expression as potential gene transfer system in Parkinson's disease

5 June 2018

1 reference

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Two transmembrane aspartates in presenilin-1 required for presenilin endoproteolysis and gamma-secretase activity

5 June 2018

1 reference

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

5 June 2018

1 reference

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

5 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3018386

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

5 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3018386

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

5 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3018386

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

5 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3018386

5 June 2018

1 reference

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Immortalized murine striatal neuronal cell lines expressing dopamine receptors and cholinergic properties

5 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3018386

Mutant huntingtin and glycogen synthase kinase 3-beta accumulate in neuronal lipid rafts of a presymptomatic knock-in mouse model of Huntington's disease

5 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3018386

Polyglutamine expansion in huntingtin increases its insertion into lipid bilayers

18 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3018386

N-terminal proteolysis of full-length mutant huntingtin in an inducible PC12 cell model of Huntington's disease

18 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3018386

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

18 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3018386

Autophagy regulates the processing of amino terminal huntingtin fragments

18 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3018386

Tissue-specific proteolysis of Huntingtin (htt) in human brain: evidence of enhanced levels of N- and C-terminal htt fragments in Huntington's disease striatum.

18 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3018386

Functional gamma-secretase inhibitors reduce beta-amyloid peptide levels in brain

18 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3018386

Huntingtin expression stimulates endosomal-lysosomal activity, endosome tubulation, and autophagy.

18 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3018386

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

18 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3018386

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

18 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3018386

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

18 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3018386

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

18 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3018386

Peptidomimetic probes and molecular modeling suggest that Alzheimer's gamma-secretase is an intramembrane-cleaving aspartyl protease

18 August 2018

1 reference

PubMed

https://pubmed.ncbi.nlm.nih.gov/21156064

inferred from PubMed ID database lookup

12 December 2020

based on heuristic

Identifiers

DOI

10.1186/1750-1326-5-58

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:21156064%20AND%20SRC:MED&resulttype=core&format=json

Dimensions Publication ID

18 January 2020

1 reference

9 September 2019

https://scigraph.springernature.com/pub.10.1186/1750-1326-5-58

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:21156064%20AND%20SRC:MED&resulttype=core&format=json

18 January 2020

1 reference