Researchers in the south of France have studied the determinants of the degeneration and fibrosis observed in cardiomyopathy linked to Duchenne muscular dystrophy (DMD) in animals and humans:
- human pluripotent stem cells from DMD patients were first transformed into cardiomyocytes,
- myocardial tissue from DMD model dogs (GRMD) was also analysed,
- dysfunction of the type 2 ryanodine receptor and disruption of calcium flow appear to be at the root of fibrosis and myocardial senescence.
The researchers were also able to demonstrate the value of a molecule (S107) in stabilising the phenomenon and improving the phenotype.
