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Article

Cerebellar soluble mutant ataxin-3 level decreases during disease progression in Spinocerebellar Ataxia Type 3 mice.

PloS one - 1 Jan 2013

Nguyen Huu Phuc, Hübener Jeannette, Weber Jonasz Jeremiasz, Grueninger Stephan, Riess Olaf, Weiss Andreas

Abstract excerpt

Spinocerebellar Ataxia Type 3 (SCA3), also known as Machado-Joseph disease, is an autosomal dominantly inherited neurodegenerative disease caused by an expanded polyglutamine stretch in the ataxin-3 protein. A pathological hallmark of the disease is cerebellar and brainstem atrophy, which correlates with the formation of intranuclear aggregates in a specific subset of neurons. Several studies have demonstrated...

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Identifiers

PMID: 23626768
DOI: 10.1371/journal.pone.0062043
PMCID: PMC3633920

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 5 Sept 2026. Manifest publication-graph-cohort-2026-09-05.1.

Source receipt: pubmed, retrieved 5 Sept 2026.

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