Abstract：
:Facioscapulohumeral muscular dystrophy (FSHD) is a disabling inherited muscular disorder characterized by asymmetric, progressive muscle weakness and degeneration. Patients display widely variable disease onset and severity, and sometimes present with extra-muscular symptoms. There is a consensus that FSHD is caused by the aberrant production of the double homeobox protein 4 (DUX4) transcription factor in skeletal muscle. DUX4 is normally expressed during early embryonic development, and is then effectively silenced in all tissues except the testis and thymus. Its reactivation in skeletal muscle disrupts numerous signalling pathways that mostly converge on cell death. Here, we review studies on DUX4-affected pathways in skeletal muscle and provide insights into how understanding these could help explain the unique pathogenesis of FSHD.
journal_name
Int J Mol Scijournal_title
International journal of molecular sciencesauthors
Lim KRQ,Nguyen Q,Yokota Tdoi
10.3390/ijms21030729subject
Has Abstractpub_date
2020-01-22 00:00:00issue
3issn
1422-0067pii
ijms21030729journal_volume
21pub_type
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