Article
DUX4-induced dsRNA and MYC mRNA stabilization activate apoptotic pathways in human cell models of facioscapulohumeral dystrophy.
PLoS genetics - 1 Mar 2017
Shadle Sean C, Zhong Jun Wen, Campbell Amy E, Conerly Melissa L, Jagannathan Sujatha, Wong Chao-Jen, Morello Timothy D, van der Maarel Silvère M, Tapscott Stephen J
Abstract excerpt
Facioscapulohumeral dystrophy (FSHD) is caused by the mis-expression of DUX4 in skeletal muscle cells. DUX4 is a transcription factor that activates genes normally associated with stem cell biology and its mis-expression in FSHD cells results in apoptosis. To identify genes and pathways necessary for DUX4-mediated apoptosis, we performed an siRNA screen in an RD rhabdomyosarcoma cell line with an inducible DUX4...
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