Researchers are hoping to find better treatments for arrhythmogenic right ventricular cardiomyopathy (ARVC) by examining how the disease develops in an affected heart cell.
The scientists at the Johns Hopkins University School of Medicine in California have created a laboratory-grown cell model of ARVC by transforming skin cells from two patients into heart cells using stem cell technology. For the first time the scientists were able to coax the cells to mature so they would mimic the disease that affects young adults.
The research has just been published online by Nature.
Dr Daniel Judge, medical director of the inherited heart disease centre at Johns Hopkins, said: With this new model, we hope we are on a path to developing better therapies for this disease.”
The disease can cause abnormal heart rhythms. Current treatments include drugs, such as beta blockers, and internal defibrillators (ICDs) to shock the heart back into a normal rhythm. Patients are usually told not to take part in competitive sport.
Dr Judge said it was difficult to show that a disease-in-a-dish model was clinically relevant for an adult disease. But researchers had made a key finding — they could repeat the defects in this disease only when they induced chemical reactions seen in an adult.
Senior author of the study Dr Huei-Sheng Vincent Chen said: “This is an important breakthrough considering that ARVC symptoms usually don’t arise until young adulthood. Yet the stem cells we’re working with are embryonic in nature.
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