Watch DUX4 switch on in facioscapulohumeral MD muscle cells

In this amazing time-lapse video, as immature muscle cells (myoblasts) develop and fuse to form elongated myotubes, the nuclei inside the cells glow green as they express DUX4 protein, a key player in FSH muscular dystrophy. This video, recorded over 118 hours, was created by Dan Miller’s lab at the University of Washington for its 2015 Human Molecular Genetics publication. The Miller team designed a “nuclear turbo” green fluorescent protein (ntGFP) engineered to cause cell nuclei to glow green when the DUX4 protein is expressed in FSHD muscle cells. As green nuclei cluster and intensify in color, you can see the host myotube disintegrate in a demonstration of DUX4’s toxicity.

Reference
Amanda M. Rickard, Lisa M. Petek and Daniel G. Miller. Endogenous DUX4 expression in FSHD myotubes is sufficient to cause cell death and disrupts RNA splicing and cell migration pathways. Hum. Mol. Genet. (2015)doi: 10.1093/hmg/ddv315. First published online:August 5, 2015. PubMed.

This work was supported by the National Institutes of Health (NIAMS AR064197-01A1), the Friends of FSH Research, the FSHD Canada Foundation, Kacy Murray and the Anderson Family Foundation. Additional supplementary material and videos are available at HMG online.