Interview Christelle Monville

Christelle Monville, Professor at Evry University

Principal Investigator “ Duchenne Muscular Dystrophy Team”

1. On which pathology is your research focused on I-Stem?

The team is interested in the treatment of the cardiopathy related to the Duchenne muscular dystrophy (DMD). This disease, which is inherited in what is known as an X-linked recessive pattern, touches a boy on 3500 birth each year in France. This disease is a rapidly-worsening form of muscular dystrophy. It is caused by a defective gene for dystrophin (a protein in the muscles) and symptoms usually appear before age 6 and may appear as early as infancy. There is progressive muscle weakness of the legs and pelvis, which is associated with a loss of muscle mass (wasting). Muscle weakness also occurs in the arms, neck, and other areas, but not as severely or as early as in the lower half of the body. Muscular weakness and skeletal deformities contribute to frequent breathing disorders. Cardiomyopathy occurs in almost all cases.

2. What are your expectations from stem cells within your research area ?

There is no known cure for Duchenne muscular dystrophy yet although recent stem-cell research is showing some ways to replace damaged muscle tissue. The heart is an organ that lacks intrinsic regenerative capcities, and in our research we are using stem cells to replace dying cardiomyocytes and restore heart function.

3. What is specifically relevant in hES cell research ?

Scientists have used a variety of cell types and examined their capacity to regenerate the myocardium and among them only stem cells seem to have the ability to differentiate into cardiomyocytes. It is now necessary to investigate their behaviour and functional characteristics of these cells after grafting.

4. Which aims are you expecting to reach within the next 10 years ?

To test the behavior of the grafted cells we have developed a model of heart organotypic culture and showed that the cells effectively differentiate into cardiomyocytes and are integrated into the host tissue. We should now show that they are able to be integrated into a pathological tissue and restore the lost cardiac functions. For that, series of experiments will be carried on GRMD dogs (Golden delicious Retriever Muscular Dystrophy), genetic model of the Duchenne myopathy, in collaboration with the team of Stephan Blot of the ENVA. The results of Michel Pucéat, Philippe Ménasché on the treatment of cardiac ischemia and our results make us hopping a clinical application in the ten years coming.


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