Author: Darko Bosnakovski; Erik A. Toso; Olivia O. Recht; Anja Cucak; Abhinav K Jain; Michelle C. Barton; Michael Kyba
Title: p53 is not necessary for DUX4 pathology Document date: 2017_3_19
ID: jopeo9gs_2
Snippet: Mechanisms of DUX4-mediated pathology are currently being investigated actively. At high levels of expression, DUX4 causes toxicity that leads to cell death of myoblasts (Bosnakovski et al. 2008; Kowaljow et al. 2007 ), while at low levels of expression, it impairs myogenic differentiation (Dandapat et al. 2014) and sensitizes cells to oxidative stress (Bosnakovski et al. 2008 ). A mouse model allowing doxycycline-regulated DUX4 expression has re.....
Document: Mechanisms of DUX4-mediated pathology are currently being investigated actively. At high levels of expression, DUX4 causes toxicity that leads to cell death of myoblasts (Bosnakovski et al. 2008; Kowaljow et al. 2007 ), while at low levels of expression, it impairs myogenic differentiation (Dandapat et al. 2014) and sensitizes cells to oxidative stress (Bosnakovski et al. 2008 ). A mouse model allowing doxycycline-regulated DUX4 expression has recapitulated these phenotypes in primary myoblasts (Dandapat et al. 2014) while also having several nonmuscle related phenotypes due to low basal levels of DUX4 expression in the absence of All rights reserved. No reuse allowed without permission.
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