A new rat model of Becker muscular dystrophy, with a deletion of exons 45-47 of the Dmd gene, has been developed by a French team including researchers from the Institute’s Center for Research in Myology :
- This model rat shows moderate impairment of locomotion and diaphragm, associated with progressive cardiomyopathy.
- Histology shows disorganisation of the intercalary disc, altering the cohesion of the cardiac syncytium and manifesting itself in electrocardiographic abnormalities.
This preclinical model provides an opportunity to study the role of dystrophin in intercellular communication, particularly at cardiac level.
