Figure 2.

The central unstructured domain of DUX4 is dispensable for transcriptional activation and cytotoxicity. (A) Scheme of DUX4 deletion mutants. HD: homeodomain. GFP was added to visualize DUX4 fragments. The number of DUX4 amino acids in the N-terminal and C-terminal parts of each construct are indicated. (B) RT-qPCR analysis of RNA of the target gene ZSCAN4 after transient transfection of each construct. Protein expression levels of DUX4 deletion mutants are shown by immune blot, below. (C) Viability of C2C12 cells expressing different levels of each DUX4 deletion mutant. All constructs were targeted into the same genomic inducible locus. Cell viability (ATP content) was measured 24 h after treatment with doxycycline.