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Article

Overexpression of mutant ataxin-3 in mouse cerebellum induces ataxia and cerebellar neuropathology.

Cerebellum (London, England) - 1 Aug 2013

Nóbrega Clévio, Nascimento-Ferreira Isabel, Onofre Isabel, Albuquerque David, Conceição Mariana, Déglon Nicole, de Almeida Luís Pereira

Abstract excerpt

Machado-Joseph disease (MJD), also known as spinocerebellar ataxia type 3 (SCA3), is a fatal, dominant neurodegenerative disorder caused by the polyglutamine-expanded protein ataxin-3. Clinical manifestations include cerebellar ataxia and pyramidal signs culminating in severe neuronal degeneration. Currently, there is no therapy able to modify disease progression. In the present study, we aimed at investigating...

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PMID: 23242710
DOI: 10.1007/s12311-012-0432-0

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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