(Q45301604)

11 December 2017

title

Inhibition of 26S proteasome activity by huntingtin filaments but not inclusion bodies isolated from mouse and human brain (English)

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11 December 2017

8

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11 December 2017

author name string

Miguel Díaz-Hernández

1

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11 December 2017

Adriana G Valera

2

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11 December 2017

María A Morán

3

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11 December 2017

Pilar Gómez-Ramos

4

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11 December 2017

Beatriz Alvarez-Castelao

5

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11 December 2017

José G Castaño

6

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11 December 2017

Félix Hernández

7

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11 December 2017

language of work or name

English

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publication date

19 June 2006

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Journal of Neurochemistry

11 December 2017

published in

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11 December 2017

volume

98

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11 December 2017

issue

5

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11 December 2017

page(s)

1585-1596

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

11 December 2017

cites work

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03968.X

Impairment of the ubiquitin-proteasome system by protein aggregation

21 January 2018

1 reference

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Global impairment of the ubiquitin-proteasome system by nuclear or cytoplasmic protein aggregates precedes inclusion body formation.

21 January 2018

1 reference

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Neuronal dysfunction in a polyglutamine disease model occurs in the absence of ubiquitin-proteasome system impairment and inversely correlates with the degree of nuclear inclusion formation

21 January 2018

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The ubiquitin-proteasome pathway: the complexity and myriad functions of proteins death

21 January 2018

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Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1.

21 January 2018

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https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03968.X

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

21 January 2018

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https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03968.X

The proteasome, a novel protease regulated by multiple mechanisms

21 January 2018

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https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03968.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03968.X

Full motor recovery despite striatal neuron loss and formation of irreversible amyloid-like inclusions in a conditional mouse model of Huntington's disease.

21 January 2018

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https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03968.X

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

21 January 2018

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Proteasomal expression, induction of immunoproteasome subunits, and local MHC class I presentation in myofibrillar myopathy and inclusion body myositis.

21 January 2018

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Testing the ubiquitin-proteasome hypothesis of neurodegeneration in vivo.

21 January 2018

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

21 January 2018

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

21 January 2018

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Aggresomes, inclusion bodies and protein aggregation

21 January 2018

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A transgenic mouse model of the ubiquitin/proteasome system

21 January 2018

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Decreased nuclear beta-catenin, tau hyperphosphorylation and neurodegeneration in GSK-3beta conditional transgenic mice

21 January 2018

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Requirement of an intact microtubule cytoskeleton for aggregation and inclusion body formation by a mutant huntingtin fragment.

21 January 2018

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ASK1 is essential for endoplasmic reticulum stress-induced neuronal cell death triggered by expanded polyglutamine repeats

21 January 2018

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Huntingtin spheroids and protofibrils as precursors in polyglutamine fibrilization

21 January 2018

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Intranuclear neuronal inclusions: a common pathogenic mechanism for glutamine-repeat neurodegenerative diseases?

21 January 2018

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Protein aggregation and neurodegenerative disease

21 January 2018

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https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03968.X

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

21 January 2018

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Cellular defenses against unfolded proteins: a cell biologist thinks about neurodegenerative diseases

21 January 2018

1 reference

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Polyglutamine-expanded androgen receptors form aggregates that sequester heat shock proteins, proteasome components and SRC-1, and are suppressed by the HDJ-2 chaperone

21 January 2018

1 reference

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From Alzheimer to Huntington: why is a structural understanding so difficult?

21 January 2018

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The ubiquitin-proteasome system in Huntington's disease.

21 January 2018

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Eukaryotic proteasomes cannot digest polyglutamine sequences and release them during degradation of polyglutamine-containing proteins

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03968.X

Aggregate formation inhibits proteasomal degradation of polyglutamine proteins.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03968.X

The 26S proteasome: a molecular machine designed for controlled proteolysis

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03968.X

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

21 January 2018

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https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03968.X

Glutamine repeats and neurodegeneration

21 January 2018

1 reference

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10.1111/J.1471-4159.2006.03968.X

21 January 2018

Identifiers

DOI

1 reference

11 December 2017

1 reference