Selected article for: "bi transgenic mouse and mouse model"
Author: Takako I. Jones; Guo-Liang Chew; Pamela Barraza-Flores; Spencer Schreier; Monique Ramirez; Ryan D. Wuebbles; Dean J. Burkin; Robert K. Bradley; Peter L. Jones
Title: Transgenic mice expressing tunable levels of DUX4 develop characteristic facioscapulohumeral muscular dystrophy-like pathophysiology ranging in severity
  • Document date: 2018_11_15
    • ID: 1yto01tr_5
    Snippet: Here we show that the FLExD mouse model, when crossed with an appropriate inducible Cre mouse, produces a bi-transgenic model with chronic low levels of DUX4-fl expression which can be induced at the desired time to develop a more severe pathology, reproducibly recapitulating many aspects of FSHD pathophysiology. Importantly, the severity of pathology can be controlled by the investigator, thus providing several suitable models for therapeutic in.....
    Document: Here we show that the FLExD mouse model, when crossed with an appropriate inducible Cre mouse, produces a bi-transgenic model with chronic low levels of DUX4-fl expression which can be induced at the desired time to develop a more severe pathology, reproducibly recapitulating many aspects of FSHD pathophysiology. Importantly, the severity of pathology can be controlled by the investigator, thus providing several suitable models for therapeutic interventions targeting DUX4-fl mRNA, protein, and certain downstream pathways.

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