Mutation of the E6-AP ubiquitin ligase reduces nuclear inclusion frequency while accelerating polyglutamine-induced pathology in SCA1 mice (Q54066632)

24 May 2018

title

Mutation of the E6-AP ubiquitin ligase reduces nuclear inclusion frequency while accelerating polyglutamine-induced pathology in SCA1 mice (English)

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24 May 2018

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7

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24 May 2018

Huda Zoghbi

9

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24 May 2018

Aaron Ciechanover

6

A Ciechanover

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24 May 2018

2

E Reinstein

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24 May 2018

Yaling Sun

3

Y Sun

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24 May 2018

Yong-Hui Jiang

5

Y Jiang

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24 May 2018

Arthur L Beaudet

8

A L Beaudet

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24 May 2018

author name string

C J Cummings

1

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24 May 2018

B Antalffy

4

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24 May 2018

publication date

1 December 1999

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24 May 2018

language of work or name

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24 May 2018

volume

24

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24 May 2018

issue

4

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24 May 2018

page(s)

879-892

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24 May 2018

Imprinted expression of the murine Angelman syndrome gene, Ube3a, in hippocampal and Purkinje neurons

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Evidence for proteasome involvement in polyglutamine disease: localization to nuclear inclusions in SCA3/MJD and suppression of polyglutamine aggregation in vitro

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Purkinje cell expression of a mutant allele of SCA1 in transgenic mice leads to disparate effects on motor behaviors, followed by a progressive cerebellar dysfunction and histological alterations.

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

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

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Cloning and expression of the cDNA for E6-AP, a protein that mediates the interaction of the human papillomavirus E6 oncoprotein with p53.

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A family of proteins structurally and functionally related to the E6-AP ubiquitin-protein ligase

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Expression of ataxin-2 in brains from normal individuals and patients with Alzheimer's disease and spinocerebellar ataxia 2.

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Mutation of the Angelman ubiquitin ligase in mice causes increased cytoplasmic p53 and deficits of contextual learning and long-term potentiation.

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Huntingtin is ubiquitinated and interacts with a specific ubiquitin-conjugating enzyme

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

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A novel ubiquitination factor, E4, is involved in multiubiquitin chain assembly.

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Involvement of the molecular chaperone Ydj1 in the ubiquitin-dependent degradation of short-lived and abnormal proteins in Saccharomyces cerevisiae

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Expansion of an unstable trinucleotide CAG repeat in spinocerebellar ataxia type 1.

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Combinatorial control in ubiquitin-dependent proteolysis: don't Skp the F-box hypothesis

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7 January 2021

Protein fate in neurodegenerative proteinopathies: polyglutamine diseases join the (mis)fold

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7 January 2021

Intranuclear inclusions of expanded polyglutamine protein in spinocerebellar ataxia type 3.

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Nuclear inclusions in glutamine repeat disorders: are they pernicious, coincidental, or beneficial?

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7 January 2021

Ataxin-1 with an expanded glutamine tract alters nuclear matrix-associated structures

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The role of E6AP in the regulation of p53 protein levels in human papillomavirus (HPV)-positive and HPV-negative cells.

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Atrophin-1, the DRPLA Gene Product, Interacts with Two Families of WW Domain-Containing Proteins

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Spinocerebellar ataxia: variable age of onset and linkage to human leukocyte antigen in a large kindred

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7 January 2021

10.1016/S0896-6273(00)81035-1

Identifiers

DOI

1 reference

24 May 2018

1 reference

24 May 2018