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

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

https://devfeature-collection.sl.nsw.gov.au/record/TN_cdi_doaj_primary_oai_doaj_org_article_c6f8f8e493e24d9098043d936bb0bb26

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

About this item

Full title

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

Publisher

England: The Company of Biologists Ltd

Journal title

Disease models & mechanisms, 2017-10, Vol.10 (10), p.1211-1216

Language

English

Formats

Publication information

Publisher

England: The Company of Biologists Ltd

More information

Scope and Contents

Contents

Facioscapulohumeral muscular dystrophy (FSHD) is a genetically dominant myopathy caused by mutations that disrupt repression of the normally silent
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...

Alternative Titles

Full title

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

Identifiers

Primary Identifiers

Record Identifier

TN_cdi_doaj_primary_oai_doaj_org_article_c6f8f8e493e24d9098043d936bb0bb26

Permalink

https://devfeature-collection.sl.nsw.gov.au/record/TN_cdi_doaj_primary_oai_doaj_org_article_c6f8f8e493e24d9098043d936bb0bb26

Other Identifiers

ISSN

1754-8403

E-ISSN

1754-8411

DOI

10.1242/dmm.030064

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