Dystrophins DP71 and DP427 determine cell viability during proliferation and myofibre differentiation - PubMed
3 hours ago
- #Duchenne muscular dystrophy
- #cell viability
- #dystrophin isoforms
- DP71 and DP427 dystrophins are key for cell viability during proliferation and myofiber differentiation, respectively, affecting disease severity in DMD.
- Absence of DP71 or DP427 leads to similar cellular defects like increased membrane permeability and ROS, but triggers distinct transcriptome changes impacting proliferation vs. structural reorganization.
- Utrophin UP395 can partially compensate for both DP427 and DP71, influencing therapeutic strategies for DMD patients with different dystrophin deficiencies.