Intrinsic Epigenetic Regulation of the D4Z4 Macrosatellite Repeat in a Transgenic Mouse Model for FSHD

Facioscapulohumeral dystrophy (FSHD) is a progressive muscular dystrophy caused by decreased epigenetic repression of the D4Z4 macrosatellite repeats and ectopic expression of DUX4, a retrogene encoding a germline transcription factor encoded in each repeat. Unaffected individuals generally have mor...

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Auteurs principaux: Krom, Yvonne D., Thijssen, Peter E., Young, Janet M., den Hamer, Bianca, Balog, Judit, Yao, Zizhen, Maves, Lisa, Snider, Lauren, Knopp, Paul, Zammit, Peter S., Rijkers, Tonnie, van Engelen, Baziel G. M., Padberg, George W., Frants, Rune R., Tawil, Rabi, Tapscott, Stephen J., van der Maarel, Silvère M.
Format: Artigo
Langue:Inglês
Publié: Public Library of Science 2013
Sujets:
Research Article
Accès en ligne:https://ncbi.nlm.nih.gov/pmc/articles/PMC3616921/
https://ncbi.nlm.nih.gov/pubmed/23593020
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1371/journal.pgen.1003415
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