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Article

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

The Journal of neuroscience : the official journal of the Society for Neuroscience - 10 Nov 2004

Goti Daniel, Katzen Scott M, Mez Jesse, Kurtis Noam, Kiluk Jennifer, Ben-Haïem Lea, Jenkins Nancy A, Copeland Neal G, Kakizuka Akira, Sharp Alan H, Ross Christopher A, Mouton Peter R, Colomer Veronica

Abstract excerpt

Machado-Joseph disease (MJD) is an inherited neurodegenerative disorder caused by ataxin-3 with a polyglutamine expansion. It is proposed that a toxic cleavage fragment of mutant ataxin-3 alternatively spliced isoform mjd1a triggers neurodegeneration, although this fragment has not yet been detec...

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Identifiers

PMID: 15537899
DOI: 10.1523/JNEUROSCI.2734-04.2004
PMCID: PMC6730179

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 1 Sept 2026. Prepared record publication-15537899-2026-09-01.1. Manifest publication-graph-cohort-2026-09-01.1.

Source receipt: PubMed via Helena Literature Corpus, retrieved 1 Sept 2026.

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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.PMID 15537899Read ↗

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