DUX4 and facioscapulohumeral muscular dystrophy: A transcriptomic analysis on whole muscle revealed that muscles from the iUDX4pA-HSA mouse model recapitulate the gene expression signature found in MRI-guided human FSHD muscle biopsies, including genes associated with cell-death signaling pathways as well as DUX4 target genes The iDUX4pA mouse model has been used to develop a therapeutic strategy by the team, showing that the p300-inhbiting molecule ip300w efficiently decreased DUX4-mediated toxicity in skeletal muscle [56].