Misfolding promotes the ubiquitination of polyglutamine-expanded ataxin-3, the defective gene product in SCA3/MJD. (Q40473493)

20 September 2017

Misfolding promotes the ubiquitination of polyglutamine-expanded ataxin-3, the defective gene product in SCA3/MJD. (English)

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20 September 2017

protein ubiquitination

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Nihar Ranjan Jana

1

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PubMed ID

15639784

20 September 2017

Nobuyuki Nukina

series ordinal

2

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20 September 2017

1 January 2004

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20 September 2017

Neurotoxicity Research

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20 September 2017

6

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20 September 2017

7-8

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20 September 2017

523-533

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https://scigraph.springernature.com/pub.10.1007/bf03033448

20 September 2017

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cites work

Machado-Joseph disease: an autosomal dominant motor system degeneration

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12 December 2020

Polyglutamine length-dependent interaction of Hsp40 and Hsp70 family chaperones with truncated N-terminal huntingtin: their role in suppression of aggregation and cellular toxicity

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12 December 2020

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

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12 December 2020

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

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12 December 2020

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

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12 December 2020

Machado-Joseph disease gene product identified in lymphocytes and brain.

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12 December 2020

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

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12 December 2020

Kennedy's disease: caspase cleavage of the androgen receptor is a crucial event in cytotoxicity

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12 December 2020

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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12 December 2020

Glutamine repeats and neurodegeneration

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12 December 2020

The complex pathology of trinucleotide repeats

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12 December 2020

A huntingtin-associated protein enriched in brain with implications for pathology

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12 December 2020

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

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12 December 2020

Nuclear localization or inclusion body formation of ataxin-2 are not necessary for SCA2 pathogenesis in mouse or human

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12 December 2020

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

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12 December 2020

CAG expansions in a novel gene for Machado-Joseph disease at chromosome 14q32.1

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12 December 2020

Expanding our understanding of polyglutamine diseases through mouse models

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12 December 2020

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

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12 December 2020

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

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12 December 2020

Incorporation of glutamine repeats makes protein oligomerize: implications for neurodegenerative diseases

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12 December 2020

SH3GL3 associates with the Huntingtin exon 1 protein and promotes the formation of polygln-containing protein aggregates

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12 December 2020

Neuronal intranuclear inclusions in polyglutamine diseases: nuclear weapons or nuclear fallout?

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12 December 2020

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

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12 December 2020

Cleavage of atrophin-1 at caspase site aspartic acid 109 modulates cytotoxicity

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12 December 2020

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

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12 December 2020

Machado-Joseph disease gene product is a cytoplasmic protein widely expressed in brain

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12 December 2020

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

12 December 2020

Ataxin-3, the MJD1 gene product, interacts with the two human homologs of yeast DNA repair protein RAD23, HHR23A and HHR23B

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12 December 2020

Inhibition of huntingtin fibrillogenesis by specific antibodies and small molecules: implications for Huntington's disease therapy

1 reference

12 December 2020

Trinucleotide repeats in neurogenetic disorders

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12 December 2020

Suppression of polyglutamine-mediated neurodegeneration in Drosophila by the molecular chaperone HSP70.

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12 December 2020

Immunohistochemical study of neuronal intranuclear and cytoplasmic inclusions in Machado-Joseph disease.

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12 December 2020

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

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12 December 2020

Fourteen and counting: unraveling trinucleotide repeat diseases

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12 December 2020

Caspase activation during apoptotic cell death induced by expanded polyglutamine in N2a cells.

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12 December 2020

Ataxin-3 with an altered conformation that exposes the polyglutamine domain is associated with the nuclear matrix

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12 December 2020

Caspase cleavage of gene products associated with triplet expansion disorders generates truncated fragments containing the polyglutamine tract

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12 December 2020

Analysis of the role of heat shock protein (Hsp) molecular chaperones in polyglutamine disease

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12 December 2020

Polyglutamine expansions: proteolysis, chaperones, and the dangers of promiscuity

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12 December 2020

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

1 reference

12 December 2020

Ataxin-3 is transported into the nucleus and associates with the nuclear matrix

1 reference

12 December 2020

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

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12 December 2020

Peptides containing glutamine repeats as substrates for transglutaminase-catalyzed cross-linking: relevance to diseases of the nervous system

1 reference

12 December 2020

Identifiers

DOI

10.1007/BF03033448

1 reference

20 September 2017

1 reference

PubMed ID

15639784