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. 2018 Dec 6;28(8):1244–1259. doi: 10.1093/hmg/ddy405

Figure 7.

Figure 7

Suppression of PGC1α/ERRα expression in FSHD and rescue by ERRα agonists. Schematic summarising that PGC1α/ERRα suppression in FSHD drives an FSHD hypotrophic phenotype that can be rescued by ERRα agonists biochanin A, daidzein or genistein. Suppression of the ERRα/PGC1α pathway in FSHD patients could also contribute to know features of FSHD pathology including oxidative stress sensitivity, aberrant vasculature and inflammation.