Medical College of Wisconsin Researcher to Study Dystrophic Cardiomyopathy

The Medical College of Wisconsin (MCW) received a five-year, $650,000 award from the National Institutes of Health’s National Heart, Lung and Blood Institute to investigate how muscular dystrophy causes cardiac muscle to deteriorate, a condition known as dystrophic cardiomyopathy.

Jennifer L. Strande, M.D., Ph.D., assistant professor of medicine – cardiovascular and a cardiologist at Froedtert & The Medical College, is the principal investigator for the grant. This grant supports a five-year career development plan. Dr. Strande’s mentor for the project is Zeljko Bosnjak, Ph.D., professor and vice chairman for research in anesthesiology at the Medical College.

Muscular dystrophy is a genetic disease in which patients suffer a progressive loss of muscle strength and function. The most common forms, Duchenne and Becker muscular dystrophy, are found in 400 to 600 newborn boys in the United States each year, according to the Centers for Disease Control and Prevention. In addition to affecting the skeletal muscles that operate voluntary movement, muscular dystrophy can also weaken heart function and threaten the patient’s cardiovascular health. Duchenne muscular dystrophy is the most common fatal genetic disease and heart failure is an emerging threat to these patients.

In this project, Dr. Strande will study the first human model of dystrophic cardiomyopathy by using heart cells derived from induced pluripotent stem cells, which are skin cells taken from patients and reprogrammed to function like stem cells. Her lab will run tests to determine how the various muscular dystrophy mutations affect heart cells. The team will also assess a possible treatment option that may help protect the heart from stress and damage in cases of muscular dystrophy.

The results of this investigation may generate new understanding about how the genetic mutations in muscular dystrophy affect muscle in the heart. Dr. Strande’s efforts may also lead to new ideas for improving the treatment of muscular dystrophy patients with cardiovascular concerns.

Dystrophy

http://www.ninds.nih.gov/disorders/md/detail_md.htm

http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0002172/


Dystrophin

http://ghr.nlm.nih.gov/gene/DMD