A European consortium of researchers has studied the biological and molecular characteristics of cardiac fibroblasts generated from induced pluripotent stem cells (IPSCs) from Duchenne muscular dystrophy (DMD) patients and control subjects:
- these supporting tissue cells do not express full-length dystrophin like muscle fibres,
- these fibroblasts show profound changes in their biochemical profiles, with activation of glycolysis to compensate for the decline in mitochondrial functions,
- they are also more sensitive to profibrotic stress.
This work provides a better understanding of the repercussions of the absence of dystrophin on the heart, particularly in terms of the development of fibrosis.
