(Q40493431)

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A mutant ataxin-3 putative-cleavage fragment in brains of Machado-Joseph disease patients and transgenic mice is cytotoxic above a critical concentration.

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https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:15537899%20AND%20SRC:MED&resulttype=core&format=json

5 January 2020

A mutant ataxin-3 putative-cleavage fragment in brains of Machado-Joseph disease patients and transgenic mice is cytotoxic above a critical concentration (English)

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https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:15537899%20AND%20SRC:MED&resulttype=core&format=json

5 January 2020

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Machado-Joseph disease

1 reference

based on heuristic

inferred from title

3

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https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:15537899%20AND%20SRC:MED&resulttype=core&format=json

5 January 2020

Christopher A Ross

11

object named as

Christopher A Ross

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5 January 2020

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Daniel Goti

1

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5 January 2020

Scott M Katzen

2

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5 January 2020

Noam Kurtis

4

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5 January 2020

Jennifer Kiluk

5

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5 January 2020

Lea Ben-Haïem

6

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5 January 2020

Akira Kakizuka

9

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5 January 2020

Alan H Sharp

10

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5 January 2020

Peter R Mouton

12

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5 January 2020

Veronica Colomer

13

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5 January 2020

Nancy A Jenkins

7

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5 January 2020

Neal G Copeland

8

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5 January 2020

language of work or name

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publication date

1 November 2004

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5 January 2020

Journal of Neuroscience

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5 January 2020

24

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5 January 2020

45

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Europe PubMed Central

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https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:15537899%20AND%20SRC:MED&resulttype=core&format=json

5 January 2020

10266-10279

1 reference

Europe PubMed Central

PMC publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:15537899%20AND%20SRC:MED&resulttype=core&format=json

5 January 2020

describes a project that uses

1 reference

Europe PubMed Central

11 June 2022

https://www.ebi.ac.uk/europepmc/webservices/rest/PMC6730179/fullTextXML

based on heuristic

inferred from PubMed Central ID database lookup

Heterogeneous intracellular localization and expression of ataxin-3.

1 reference

https://pubmed.ncbi.nlm.nih.gov/15537899

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/15537899

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Inhibition of caspase-1 slows disease progression in a mouse model of Huntington's disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/15537899

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Catalytic properties of the caspases

1 reference

https://pubmed.ncbi.nlm.nih.gov/15537899

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Cell cycle arrest enhances the in vitro cellular toxicity of the truncated Machado-Joseph disease gene product with an expanded polyglutamine stretch

1 reference

https://pubmed.ncbi.nlm.nih.gov/15537899

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Nuclear accumulation of truncated atrophin-1 fragments in a transgenic mouse model of DRPLA.

1 reference

https://pubmed.ncbi.nlm.nih.gov/15537899

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Characterization of the rat spinocerebellar ataxia type 3 gene

1 reference

https://pubmed.ncbi.nlm.nih.gov/15537899

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Amino-terminal fragments of mutant huntingtin show selective accumulation in striatal neurons and synaptic toxicity

1 reference

https://pubmed.ncbi.nlm.nih.gov/15537899

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

The polyglutamine expansion in spinocerebellar ataxia type 6 causes a beta subunit-specific enhanced activation of P/Q-type calcium channels in Xenopus oocytes.

1 reference

https://pubmed.ncbi.nlm.nih.gov/15537899

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Ataxin-3 is translocated into the nucleus for the formation of intranuclear inclusions in normal and Machado-Joseph disease brains

1 reference

https://pubmed.ncbi.nlm.nih.gov/15537899

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/15537899

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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://pubmed.ncbi.nlm.nih.gov/15537899

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Involvement of the cerebral cortex and autonomic ganglia in Machado-Joseph disease.

1 reference

https://pubmed.ncbi.nlm.nih.gov/15537899

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Beyond the Qs in the polyglutamine diseases

1 reference

https://pubmed.ncbi.nlm.nih.gov/15537899

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Genetic ablation of orexin neurons in mice results in narcolepsy, hypophagia, and obesity.

1 reference

https://pubmed.ncbi.nlm.nih.gov/15537899

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

The genomic structure and expression of MJD, the Machado-Joseph disease gene

1 reference

https://pubmed.ncbi.nlm.nih.gov/15537899

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Isolation of neuronal cells with high processing activity for the Machado-Joseph disease protein

1 reference

https://pubmed.ncbi.nlm.nih.gov/15537899

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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://pubmed.ncbi.nlm.nih.gov/15537899

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

A survey of spinocerebellar ataxia in South Brazil - 66 new cases with Machado-Joseph disease, SCA7, SCA8, or unidentified disease-causing mutations.

1 reference

https://pubmed.ncbi.nlm.nih.gov/15537899

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

YAC transgenic mice carrying pathological alleles of the MJD1 locus exhibit a mild and slowly progressive cerebellar deficit.

1 reference

https://pubmed.ncbi.nlm.nih.gov/15537899

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Stereological length estimation using spherical probes.

1 reference

https://pubmed.ncbi.nlm.nih.gov/15537899

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Calpain activation in Huntington's disease.

1 reference

https://pubmed.ncbi.nlm.nih.gov/15537899

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Polyglutamine-expanded ataxin-7 promotes non-cell-autonomous purkinje cell degeneration and displays proteolytic cleavage in ataxic transgenic mice.

1 reference

https://pubmed.ncbi.nlm.nih.gov/15537899

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Huntingtin spheroids and protofibrils as precursors in polyglutamine fibrilization

1 reference

https://pubmed.ncbi.nlm.nih.gov/15537899

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/15537899

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Caspase cleavage of mutant huntingtin precedes neurodegeneration in Huntington's disease.

1 reference

https://pubmed.ncbi.nlm.nih.gov/15537899

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Polyglutamine repeat length-dependent proteolysis of huntingtin

1 reference

https://pubmed.ncbi.nlm.nih.gov/15537899

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Inhibition of calpain cleavage of huntingtin reduces toxicity: accumulation of calpain/caspase fragments in the nucleus

1 reference

https://pubmed.ncbi.nlm.nih.gov/15537899

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Caspase-mediated proteolysis of the polyglutamine disease protein ataxin-3.

1 reference

https://pubmed.ncbi.nlm.nih.gov/15537899

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

The isolation of mossy fibre endings from the granular layer of the cerebellar cortex

1 reference

https://pubmed.ncbi.nlm.nih.gov/15537899

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Nuclei from rat liver: isolation method that combines purity with high yield.

1 reference

https://pubmed.ncbi.nlm.nih.gov/15537899

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Machado-Joseph-Azorean disease. A ten-year study.

1 reference

https://pubmed.ncbi.nlm.nih.gov/15537899

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/15537899

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

SCA1 transgenic mice: A model for neurodegeneration caused by an expanded CAG trinucleotide repeat

1 reference

https://pubmed.ncbi.nlm.nih.gov/15537899

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Machado-Joseph disease mutations as the genetic basis of most spinocerebellar ataxias in Germany

1 reference

https://pubmed.ncbi.nlm.nih.gov/15537899

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Molecular and clinical correlations in spinocerebellar ataxia type 3 and Machado-Joseph disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/15537899

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Machado-Joseph disease.

1 reference

https://pubmed.ncbi.nlm.nih.gov/15537899

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Widespread expression of Huntington's disease gene (IT15) protein product

1 reference

https://pubmed.ncbi.nlm.nih.gov/15537899

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/15537899

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Spinocerebellar ataxia 3 and Machado-Joseph disease: clinical, molecular, and neuropathological features.

1 reference

https://pubmed.ncbi.nlm.nih.gov/15537899

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Expanded polyglutamine in the Machado-Joseph disease protein induces cell death in vitro and in vivo

1 reference

https://pubmed.ncbi.nlm.nih.gov/15537899

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/15537899

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Regional and cellular expression of the Machado-Joseph disease gene in brains of normal and affected individuals.

1 reference

https://pubmed.ncbi.nlm.nih.gov/15537899

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Autosomal dominant cerebellar ataxia (SCA6) associated with small polyglutamine expansions in the alpha 1A-voltage-dependent calcium channel

1 reference

https://pubmed.ncbi.nlm.nih.gov/15537899

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

A vector for expressing foreign genes in the brains and hearts of transgenic mice

1 reference

https://pubmed.ncbi.nlm.nih.gov/15537899

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/15537899

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/15537899

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Machado-Joseph disease: cerebellar ataxia and autonomic dysfunction in a patient with the shortest known expanded allele (56 CAG repeat units) of the MJD1 gene

1 reference

https://pubmed.ncbi.nlm.nih.gov/15537899

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/15537899

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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.

1 reference

https://pubmed.ncbi.nlm.nih.gov/15537899

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/15537899

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Truncated forms of the androgen receptor are associated with polyglutamine expansion in X-linked spinal and bulbar muscular atrophy

1 reference

https://pubmed.ncbi.nlm.nih.gov/15537899

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Cleavage, aggregation and toxicity of the expanded androgen receptor in spinal and bulbar muscular atrophy

1 reference

https://pubmed.ncbi.nlm.nih.gov/15537899

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/15537899

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/15537899

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Analysis of SCA1, DRPLA, MJD, SCA2, and SCA6 CAG repeats in 48 Portuguese ataxia families.

1 reference

https://pubmed.ncbi.nlm.nih.gov/15537899

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Expanded polyglutamine protein forms nuclear inclusions and causes neural degeneration in Drosophila

1 reference

https://pubmed.ncbi.nlm.nih.gov/15537899

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/15537899

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/15537899

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Identifiers

10.1523/JNEUROSCI.2734-04.2004

1 reference

Europe PubMed Central

PMC publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:15537899%20AND%20SRC:MED&resulttype=core&format=json

5 January 2020

PMC publication ID

1 reference

Europe PubMed Central

PMC publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:15537899%20AND%20SRC:MED&resulttype=core&format=json

5 January 2020

PubMed publication ID

1 reference

Europe PubMed Central

PMC publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:15537899%20AND%20SRC:MED&resulttype=core&format=json

5 January 2020

ResearchGate publication ID

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