Physiological and pathophysiological characteristics of ataxin-3 isoforms (Q64042586)

object named as

Felix von Zweydorf

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author name string

Daniel Weishäupl

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Juliane Schneider

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Barbara Peixoto Pinheiro

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Corinna Ruess

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Sandra Maria Dold

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Christian Johannes Gloeckner

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Jana Schmidt

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Thorsten Schmidt

Journal of Biological Chemistry

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language of work or name

English

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publication date

11 January 2019

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published in

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volume

294

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issue

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page(s)

644-661

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cites work

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

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An isoform of ataxin-3 accumulates in the nucleus of neuronal cells in affected brain regions of SCA3 patients.

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Heterogeneous intracellular localization and expression of ataxin-3.

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The polyglutamine neurodegenerative protein ataxin-3 binds polyubiquitylated proteins and has ubiquitin protease activity

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Genomic structure, promoter activity, and developmental expression of the mouse homologue of the Machado-Joseph disease (MJD) gene

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The effects of the polyglutamine repeat protein ataxin-1 on the UbL-UBA protein A1Up

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Ataxin-3 suppresses polyglutamine neurodegeneration in Drosophila by a ubiquitin-associated mechanism.

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Ataxin-3 is subject to autolytic cleavage.

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Ataxin-3 represses transcription via chromatin binding, interaction with histone deacetylase 3, and histone deacetylation

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Functional genomics and biochemical characterization of the C. elegans orthologue of the Machado-Joseph disease protein ataxin-3.

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p45, an ATPase subunit of the 19S proteasome, targets the polyglutamine disease protein ataxin-3 to the proteasome.

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Transient arrest in proteasomal degradation during inhibition of translation in the unfolded protein response

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Nuclear localization of ataxin-3 is required for the manifestation of symptoms in SCA3: in vivo evidence

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Cellular turnover of the polyglutamine disease protein ataxin-3 is regulated by its catalytic activity

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Josephin domain-containing proteins from a variety of species are active de-ubiquitination enzymes

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Inactivation of the mouse Atxn3 (ataxin-3) gene increases protein ubiquitination

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Opposing effects of polyglutamine expansion on native protein complexes contribute to SCA1

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Study of subcellular localization and proteolysis of ataxin-3

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The deubiquitinating enzyme ataxin-3, a polyglutamine disease protein, edits Lys63 linkages in mixed linkage ubiquitin chains.

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Early symptoms in spinocerebellar ataxia type 1, 2, 3, and 6.

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Ubiquitination directly enhances activity of the deubiquitinating enzyme ataxin-3

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CK2-dependent phosphorylation determines cellular localization and stability of ataxin-3.

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Identification and functional dissection of localization signals within ataxin-3.

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Reversibility of symptoms in a conditional mouse model of spinocerebellar ataxia type 3

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Increased transcript diversity: novel splicing variants of Machado-Joseph disease gene (ATXN3).

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Polyglutamine-induced neurodegeneration in SCA3 is not mitigated by non-expanded ataxin-3: conclusions from double-transgenic mouse models

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The Machado-Joseph disease-associated mutant form of ataxin-3 regulates parkin ubiquitination and stability

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Splice Isoforms of the Polyglutamine Disease Protein Ataxin-3 Exhibit Similar Enzymatic yet Different Aggregation Properties

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Excitation-induced ataxin-3 aggregation in neurons from patients with Machado-Joseph disease

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Calpain-mediated ataxin-3 cleavage in the molecular pathogenesis of spinocerebellar ataxia type 3 (SCA3).

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SUMO-1 modification on K166 of polyQ-expanded ataxin-3 strengthens its stability and increases its cytotoxicity

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Ubiquitination regulates the neuroprotective function of the deubiquitinase ataxin-3 in vivo.

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Ubiquitin-binding site 2 of ataxin-3 prevents its proteasomal degradation by interacting with Rad23

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12 December 2020

A knockin mouse model of spinocerebellar ataxia type 3 exhibits prominent aggregate pathology and aberrant splicing of the disease gene transcript.

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12 December 2020

The deubiquitinase ataxin-3 requires Rad23 and DnaJ-1 for its neuroprotective role in Drosophila melanogaster

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12 December 2020

mTOR inhibition activates overall protein degradation by the ubiquitin proteasome system as well as by autophagy

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Polyglutamine expansion of ataxin-3 alters its degree of ubiquitination and phosphorylation at specific sites.

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12 December 2020

Interaction of the polyglutamine protein ataxin-3 with Rad23 regulates toxicity in Drosophila models of Spinocerebellar Ataxia Type 3.

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12 December 2020

A combinatorial approach to identify calpain cleavage sites in the Machado-Joseph disease protein ataxin-3.

1 reference

12 December 2020

Karyopherin α-3 is a key protein in the pathogenesis of spinocerebellar ataxia type 3 controlling the nuclear localization of ataxin-3.

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12 December 2020

Mitochondrial Morphology, Function and Homeostasis Are Impaired by Expression of an N-terminal Calpain Cleavage Fragment of Ataxin-3

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12 December 2020