Muscle pathology from stochastic low level DUX4 expression in an FSHD mouse model
oleh: Darko Bosnakovski, Sunny S. K. Chan, Olivia O. Recht, Lynn M. Hartweck, Collin J. Gustafson, Laura L. Athman, Dawn A. Lowe, Michael Kyba
| Format: | Article |
|---|---|
| Diterbitkan: | Nature Portfolio 2017-09-01 |
Deskripsi
Facioscapulohumeral muscular dystrophy is a severe myopathy that is caused by abnormal activation of DUX4, and for which a suitable mouse model does not exist. Here, the authors generate a novel mouse model with titratable expression of DUX4, and show that it recapitulates several features of the human pathology.