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Article

Biphasic myopathic phenotype of mouse DUX, an ORF within conserved FSHD-related repeats.

PloS one - 16 Sept 2009

Bosnakovski Darko, Daughters Randy S, Xu Zhaohui, Slack Jonathan M W, Kyba Michael

Abstract excerpt

Facioscapulohumeral muscular dystrophy (FSHD) is caused by contractions of D4Z4 repeats at 4q35.2 thought to induce misregulation of nearby genes, one of which, DUX4, is actually localized within each repeat. A conserved ORF (mDUX), embedded within D4Z4-like repeats, encoding a double-homeodomain...

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Source

Open in PubMed Open full text

Identifiers

PMID: 19756142
DOI: 10.1371/journal.pone.0007003
PMCID: PMC2737622

Genes

DUX4

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 2 Sept 2026. Prepared record publication-19756142-2026-09-02.2. Manifest publication-graph-cohort-2026-09-02.2.

Source receipt: PubMed via Helena Literature Corpus, retrieved 2 Sept 2026.

Related research

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Biphasic myopathic phenotype of mouse DUX, an ORF within conserved FSHD-related repeats.PMID 19756142Read ↗

Dominant lethal pathologies in male mice engineered to contain an X-linked DUX4 transgene.Semantic proximity Read ↗

<i>DUX4</i>, a candidate gene for facioscapulohumeral muscular dystrophy, causes p53‐dependent myopathy in vivoSemantic proximity Read ↗

Mouse Dux is myotoxic and shares partial functional homology with its human paralog DUX4Semantic proximity Read ↗

An isogenetic myoblast expression screen identifies DUX4-mediated FSHD-associated molecular pathologies.Semantic proximity Read ↗

Muscle pathology from stochastic low level DUX4 expression in an FSHD mouse model.Semantic proximity Read ↗

DUX4 induces a transcriptome more characteristic of a less-differentiated cell state and inhibits myogenesisSemantic proximity Read ↗

Transgenic mouse models for investigating human <i>DUX4</i> expression during development and its roles in FSHD pathophysiologySemantic proximity Read ↗

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