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. 2015 Jan 15;2(2):151–166. doi: 10.1002/acn3.158

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© 2015 The Authors. Annals of Clinical and Translational Neurology published by Wiley Periodicals, Inc on behalf of American Neurological Association.

This is an open access article under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non-commercial and no modifications or adaptations are made.

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Exogenous DUX4-FL increased caspase activation. (A–H) DUX4-FL (red) was expressed in proliferating human myoblasts (A–D) at 24 h after plasmid transfection and in differentiated myotubes (E–H) at 48 h after BacMam vector addition. Cultures were established with myoblasts from a healthy donor. Immunostaining for activated caspase-3 (green) was found in a fraction of myoblasts and myotubes that expressed exogenous DUX4-FL (arrows). Yellow arrowhead in (A–D) indicates a DUX4-FL-positive myoblast without caspase-3 staining. (I) Caspase 3/7 enzymatic activity was increased by BacMam-mediated expression of DUX4-FL, but not DUX4-S, in both proliferating (myoblast) and differentiated (myotube) cultures of human myogenic cells. Error bars = SE, n = 4. *P < 0.05; **P < 0.01.