Huntingtin proteolysis releases non-polyQ fragments that cause toxicity through dynamin 1 dysregulation. (Q36098669)

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19 February 2020

title

Huntingtin proteolysis releases non-polyQ fragments that cause toxicity through dynamin 1 dysregulation (English)

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19 February 2020

1 reference

Marie-Thérèse El-Daher

1

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19 February 2020

Sandrine Lévêque-Fort

11

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19 February 2020

Sandrine Humbert

13

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19 February 2020

Frédéric Saudou

14

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19 February 2020

author name string

Emilie Hangen

2

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19 February 2020

Julie Bruyère

3

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19 February 2020

Ghislaine Poizat

4

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19 February 2020

Ismael Al-Ramahi

5

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19 February 2020

Raul Pardo

6

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19 February 2020

Nicolas Bourg

7

1 reference

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19 February 2020

Sylvie Souquere

8

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19 February 2020

Céline Mayet

9

1 reference

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19 February 2020

Gérard Pierron

10

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19 February 2020

Juan Botas

12

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19 February 2020

language of work or name

English

0 references

publication date

12 July 2015

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19 February 2020

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19 February 2020

volume

34

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19 February 2020

issue

17

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19 February 2020

page(s)

2255-2271

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Potential function for the Huntingtin protein as a scaffold for selective autophagy

19 February 2020

cites work

1 reference

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Huntingtin's function in axonal transport is conserved in Drosophila melanogaster

2 October 2017

1 reference

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ER structure and function

2 October 2017

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

2 October 2017

1 reference

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The Huntington disease protein accelerates breast tumour development and metastasis through ErbB2/HER2 signalling

2 October 2017

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Analysis of proteolytic processes and enzymatic activities in the generation of huntingtin n-terminal fragments in an HEK293 cell model.

2 October 2017

1 reference

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Caspase-6 activity in a BACHD mouse modulates steady-state levels of mutant huntingtin protein but is not necessary for production of a 586 amino acid proteolytic fragment.

2 October 2017

1 reference

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

2 October 2017

1 reference

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Identification of CSPα clients reveals a role in dynamin 1 regulation.

2 October 2017

1 reference

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Dynamin, a membrane-remodelling GTPase

2 October 2017

1 reference

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Cysteine proteases bleomycin hydrolase and cathepsin Z mediate N-terminal proteolysis and toxicity of mutant huntingtin

2 October 2017

1 reference

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Proteasomal dysfunction in aging and Huntington disease

2 October 2017

1 reference

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Activation of specific apoptotic caspases with an engineered small-molecule-activated protease

2 October 2017

1 reference

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Huntingtin is required for mitotic spindle orientation and mammalian neurogenesis

2 October 2017

1 reference

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Matrix Metalloproteinases Are Modifiers of Huntingtin Proteolysis and Toxicity in Huntington's Disease

2 October 2017

1 reference

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pARIS-htt: an optimised expression platform to study huntingtin reveals functional domains required for vesicular trafficking

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4585462

Proteolysis of mutant huntingtin produces an exon 1 fragment that accumulates as an aggregated protein in neuronal nuclei in Huntington 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=4585462

A class of dynamin-like GTPases involved in the generation of the tubular ER network

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4585462

Homotypic fusion of ER membranes requires the dynamin-like GTPase atlastin

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4585462

Detection of alpha-rod protein repeats using a neural network and application to huntingtin.

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4585462

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

2 October 2017

1 reference

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

2 October 2017

1 reference

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Huntington's disease: from pathology and genetics to potential therapies

2 October 2017

1 reference

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Suppression of neurodegeneration and increased neurotransmission caused by expanded full-length huntingtin accumulating in the cytoplasm

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4585462

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

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4585462

Huntingtin facilitates dynein/dynactin-mediated vesicle transport.

2 October 2017

1 reference

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Huntingtin interacting proteins are genetic modifiers of neurodegeneration

2 October 2017

1 reference

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Huntington's disease: from huntingtin function and dysfunction to therapeutic strategies.

2 October 2017

1 reference

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

2 October 2017

1 reference

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Huntingtin-HAP40 complex is a novel Rab5 effector that regulates early endosome motility and is up-regulated in Huntington's disease

2 October 2017

1 reference

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

2 October 2017

1 reference

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Normal huntingtin function: an alternative approach to Huntington's disease

2 October 2017

1 reference

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Huntingtin and the molecular pathogenesis of Huntington's disease. Fourth in molecular medicine review series

2 October 2017

1 reference

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Inhibition of calpain cleavage of huntingtin reduces toxicity: accumulation of calpain/caspase fragments in the nucleus

2 October 2017

1 reference

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Cytoplasmic aggregates trap polyglutamine-containing proteins and block axonal transport in a Drosophila model of Huntington's disease.

2 October 2017

1 reference

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Transcriptional abnormalities in Huntington disease

2 October 2017

1 reference

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The predominantly HEAT-like motif structure of huntingtin and its association and coincident nuclear entry with dorsal, an NF-kB/Rel/dorsal family transcription factor

2 October 2017

1 reference

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Caspase cleavage of mutant huntingtin precedes neurodegeneration in 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=4585462

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

2 October 2017

1 reference

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Calpain activation in 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=4585462

Huntingtin is present in the nucleus, interacts with the transcriptional corepressor C-terminal binding protein, and represses transcription

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4585462

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.

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4585462

Inhibiting caspase cleavage of huntingtin reduces toxicity and aggregate formation in neuronal and nonneuronal 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=4585462

Huntingtin interacts with a family of WW domain proteins

2 October 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4585462

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

2 October 2017

1 reference

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Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic 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=4585462

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

2 October 2017

1 reference

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

2 October 2017

1 reference

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Reticulophagy and ribophagy: regulated degradation of protein production factories.

2 October 2017

1 reference

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Autophagosome-like vacuole formation in Huntington's disease lymphoblasts

2 July 2018

1 reference

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Length-dependent gametic CAG repeat instability in the Huntington's disease knock-in mouse

2 July 2018

1 reference

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The scaffold protein Atg11 recruits fission machinery to drive selective mitochondria degradation by autophagy

2 July 2018

1 reference

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MITOL regulates endoplasmic reticulum-mitochondria contacts via Mitofusin2.

23 September 2018

1 reference

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TEV protease-mediated cleavage in Drosophila as a tool to analyze protein functions in living organisms

23 September 2018

1 reference

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Cell-type-specific TEV protease cleavage reveals cohesin functions in Drosophila neurons

23 September 2018

1 reference

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

23 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4585462

Specific caspase interactions and amplification are involved in selective neuronal vulnerability in 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=4585462

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.

23 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4585462

Dominant phenotypes produced by the HD mutation in STHdh(Q111) striatal cells

23 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4585462

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

23 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4585462

23 September 2018

Identifiers

DOI

10.15252/EMBJ.201490808

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26165689%20AND%20SRC:MED&resulttype=core&format=json

19 February 2020

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26165689%20AND%20SRC:MED&resulttype=core&format=json

19 February 2020

PubMed publication ID

26165689

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26165689%20AND%20SRC:MED&resulttype=core&format=json