Proteases acting on mutant huntingtin generate cleaved products that differentially build up cytoplasmic and nuclear inclusions (Q40709427)

23 September 2017

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

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23 September 2017

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23 September 2017

Yvon Trottier

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Jean-Louis Mandel

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object named as

Jean-Louis Mandel

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23 September 2017

Bernhard Landwehrmeyer

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G Bernhard Landwehrmeyer

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23 September 2017

Astrid Lunkes

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23 September 2017

Katrin S Lindenberg

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23 September 2017

Léa Ben-Haïem

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23 September 2017

Chantal Weber

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23 September 2017

1 August 2002

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23 September 2017

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23 September 2017

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23 September 2017

259-269

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23 September 2017

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The MEROPS database as a protease information system

23 September 2017

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https://api.crossref.org/works/10.1016%2FS1097-2765%2802%2900602-0

7 January 2021

Impairment of the ubiquitin-proteasome system by protein aggregation

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https://api.crossref.org/works/10.1016%2FS1097-2765%2802%2900602-0

7 January 2021

A masked NES in INI1/hSNF5 mediates hCRM1-dependent nuclear export: implications for tumorigenesis

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https://api.crossref.org/works/10.1016%2FS1097-2765%2802%2900602-0

7 January 2021

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

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https://api.crossref.org/works/10.1016%2FS1097-2765%2802%2900602-0

7 January 2021

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

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https://api.crossref.org/works/10.1016%2FS1097-2765%2802%2900602-0

7 January 2021

Analysis of the subcellular localization of huntingtin with a set of rabbit polyclonal antibodies in cultured mammalian cells of neuronal origin: comparison with the distribution of huntingtin in Huntington's disease autopsy brain

1 reference

https://api.crossref.org/works/10.1016%2FS1097-2765%2802%2900602-0

7 January 2021

Cleavage site for sterol-regulated protease localized to a leu-Ser bond in the lumenal loop of sterol regulatory element-binding protein-2.

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https://api.crossref.org/works/10.1016%2FS1097-2765%2802%2900602-0

7 January 2021

Second-site cleavage in sterol regulatory element-binding protein occurs at transmembrane junction as determined by cysteine panning

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https://api.crossref.org/works/10.1016%2FS1097-2765%2802%2900602-0

7 January 2021

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

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https://api.crossref.org/works/10.1016%2FS1097-2765%2802%2900602-0

7 January 2021

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

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https://api.crossref.org/works/10.1016%2FS1097-2765%2802%2900602-0

7 January 2021

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

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https://api.crossref.org/works/10.1016%2FS1097-2765%2802%2900602-0

7 January 2021

In vitro evidence for both the nucleus and cytoplasm as subcellular sites of pathogenesis in Huntington's disease.

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https://api.crossref.org/works/10.1016%2FS1097-2765%2802%2900602-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1097-2765%2802%2900602-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1097-2765%2802%2900602-0

7 January 2021

Altered proteasomal function due to the expression of polyglutamine-expanded truncated N-terminal huntingtin induces apoptosis by caspase activation through mitochondrial cytochrome c release

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https://api.crossref.org/works/10.1016%2FS1097-2765%2802%2900602-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1097-2765%2802%2900602-0

7 January 2021

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.

1 reference

https://api.crossref.org/works/10.1016%2FS1097-2765%2802%2900602-0

7 January 2021

Ataxin-1 nuclear localization and aggregation: role in polyglutamine-induced disease in SCA1 transgenic mice.

1 reference

https://api.crossref.org/works/10.1016%2FS1097-2765%2802%2900602-0

7 January 2021

Mechanism of the cleavage specificity of Alzheimer's disease gamma-secretase identified by phenylalanine-scanning mutagenesis of the transmembrane domain of the amyloid precursor protein

1 reference

https://api.crossref.org/works/10.1016%2FS1097-2765%2802%2900602-0

7 January 2021

A cellular model that recapitulates major pathogenic steps of Huntington's disease

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https://api.crossref.org/works/10.1016%2FS1097-2765%2802%2900602-0

7 January 2021

Properties of polyglutamine expansion in vitro and in a cellular model for Huntington's disease.

1 reference

https://api.crossref.org/works/10.1016%2FS1097-2765%2802%2900602-0

7 January 2021

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.

1 reference

https://api.crossref.org/works/10.1016%2FS1097-2765%2802%2900602-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1097-2765%2802%2900602-0

7 January 2021

Recruitment and the role of nuclear localization in polyglutamine-mediated aggregation

1 reference

https://api.crossref.org/works/10.1016%2FS1097-2765%2802%2900602-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1097-2765%2802%2900602-0

7 January 2021

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

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https://api.crossref.org/works/10.1016%2FS1097-2765%2802%2900602-0

7 January 2021

Beta-amyloid precursor protein cleavage by a membrane-bound protease

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https://api.crossref.org/works/10.1016%2FS1097-2765%2802%2900602-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1097-2765%2802%2900602-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1097-2765%2802%2900602-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1097-2765%2802%2900602-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1097-2765%2802%2900602-0

7 January 2021

Neuropathological classification of Huntington's disease

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https://api.crossref.org/works/10.1016%2FS1097-2765%2802%2900602-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1097-2765%2802%2900602-0

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS1097-2765%2802%2900602-0

7 January 2021

Reversal of neuropathology and motor dysfunction in a conditional model of Huntington's disease

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https://api.crossref.org/works/10.1016%2FS1097-2765%2802%2900602-0

7 January 2021

Glutamine repeats and neurodegeneration

1 reference

https://api.crossref.org/works/10.1016%2FS1097-2765%2802%2900602-0

7 January 2021

10.1016/S1097-2765(02)00602-0

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DOI

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23 September 2017

1 reference