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Article

Limited Effect of Chronic Valproic Acid Treatment in a Mouse Model of Machado-Joseph Disease.

PloS one - 1 Jan 2015

Esteves Sofia, Duarte-Silva Sara, Naia Luana, Neves-Carvalho Andreia, Teixeira-Castro Andreia, Rego Ana Cristina, Silva-Fernandes Anabela, Maciel Patrícia

Abstract excerpt

Machado-Joseph disease (MJD) is an inherited neurodegenerative disease, caused by a CAG repeat expansion within the coding region of ATXN3 gene, and which currently lacks effective treatment. In this work we tested the therapeutic efficacy of chronic treatment with valproic acid (VPA) (200mg/kg), a compound with known neuroprotection activity, and previously shown to be effective in cell, fly and nematode models...

Topics

Animals
Astrocytes
Ataxin-3
Brain
Disease Models, Animal
Endoplasmic Reticulum Chaperone BiP
Heat-Shock Proteins
Humans
Machado-Joseph Disease
Mice
Mutation

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Trinucleotide Repeat Expansion

Valproic Acid

Source

Open in PubMed Open full text

Identifiers

PMID: 26505994
DOI: 10.1371/journal.pone.0141610
PMCID: PMC4624233

Genes

ATXN3

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 31 Aug 2026.

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