In studying Marfan syndrome and related disorders at the molecular level, Dietz and his team have found that the lack of a certain connective-tissue protein adversely affects the development of various body tissues. They also made the key discovery that this condition is responsive to medication. Up until then, physicians had believed that manifestations of these disorders would be difficult or impossible to modify.
Dietz's research has led to a clinical trial using the blood pressure drug losartan in Marfan syndrome patients at risk for aortic aneurysm, which currently is taking place at 20 sites in the United States, Canada and Belgium. Preliminary testing on mice in Dietz's lab and in a smaller group of human subjects indicated that the drug dramatically slows the enlargement of the aorta, though Dietz has cautioned that full results of the broader trial will be needed to assess losartan's efficacy.
Using this new knowledge as a foundation, Dietz's team and other scientists around the world are positioned to find new treatments for not only Marfan patients, but also for disorders such as aortic aneurysm, pulmonary emphysema, fibrosis, heart valve disease and skeletal muscle myopathy, to name a few.
Dietz, who is the Victor A. McKusick Professor of Genetics and Medicine at Johns Hopkins, as well as assistant director of the school's medical genetics training program, said the prize will spotlight the importance of translational medical research.
"I am deeply honored to receive the Taubman Prize and equally thrilled by the prospect that this high-profile mechanism to recognize excellence in translational science will stimulate both interest and progress in this important field," he said.
A national selection committee reviewed more than 30 nominations for the
|Contact: Kara Gavin|
University of Michigan Health System