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Article

p53-independent DUX4 pathology in cell and animal models of facioscapulohumeral muscular dystrophy.

Disease models & mechanisms - 1 Oct 2017

Bosnakovski Darko, Gearhart Micah D, Toso Erik A, Recht Olivia O, Cucak Anja, Jain Abhinav K, Barton Michelle C, Kyba Michael

Abstract excerpt

Facioscapulohumeral muscular dystrophy (FSHD) is a genetically dominant myopathy caused by mutations that disrupt repression of the normally silent DUX4 gene, which encodes a transcription factor that has been shown to interfere with myogenesis when misexpressed at very low levels in myoblasts and to cause cell death when overexpressed at high levels. A previous report using adeno-associated virus to deliver high...

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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Source

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Identifiers

PMID: 28754837
DOI: 10.1242/dmm.030064
PMCID: PMC5665455

Genes

DUX4

Provenance

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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