A cre-inducible DUX4 transgenic mouse model for investigating facioscapulohumeral muscular dystrophy

@inproceedings{Jones2018ACD,
  title={A cre-inducible DUX4 transgenic mouse model for investigating facioscapulohumeral muscular dystrophy},
  author={Takako Iida Jones and Peter L. Jones},
  booktitle={PloS one},
  year={2018}
}
The Double homeobox 4 (DUX4) gene is an important regulator of early human development and its aberrant expression is causal for facioscapulohumeral muscular dystrophy (FSHD). The DUX4-full length (DUX4-fl) mRNA splice isoform encodes a transcriptional activator; however, DUX4 and its unique DNA binding preferences are specific to old-world primates. Regardless, the somatic cytotoxicity caused by DUX4 expression is conserved when expressed in cells and animals ranging from fly to mouse. Thus… CONTINUE READING
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