Deficiency in dystrophin leads to death with cardiorespiratory failure in humans, but mice lacking dystrophin have minimal heart defects. Blau and colleagues find that mice that lack dystrophin and have shorter telomeres exhibit cardiac defects similar to human patients, with an increase in oxidative stress. The authors also found that onset of cardiac defects could be delayed by antioxidant treatments and that, strikingly, patients also have shortened telomeres.
- Foteini Mourkioti
- Jackie Kustan
- Helen M. Blau
