Taubman Institute awards inaugural $100,000 Taubman Prize to Johns Hopkins geneticist

Harry Dietz, M.D

ANN ARBOR, Mich. — The inaugural $100,000 Taubman Prize for Excellence in Translational Medical Science has been awarded to Harry Dietz, M.D., of the Johns Hopkins University, the University of Michigan’s A. Alfred Taubman Medical Research Institute announced today.

Dietz, a cardiologist and genetics researcher, has made groundbreaking progress in the understanding of aortic aneurysm and related tissue disorders. His lab is the first to determine that some defects in the human body’s connective tissue, long thought to be unchangeable, can be modified with medication. The discovery has overturned decades of conventional wisdom and has tremendous implications for the treatment of genetic connective tissue disorders.

Dietz will receive the $100,000 grant to further his research and will appear as keynote speaker Oct. 11, 2012 at the Taubman Institute’s annual symposium in Ann Arbor.

“Dr. Dietz epitomizes translational research,” said David Ginsburg, M.D., a Taubman Scholar and member of the Taubman Prize selection committee.  “He initiated a line of research into the molecular basis for an important class of human diseases at a time when the cause was completely unknown.

“His landmark body of research, beginning at the basic laboratory bench, identified the responsible gene and developed a mouse model, leading to remarkable new insight into molecular pathogenesis. Based on these laboratory findings, Dr. Dietz then led a clinical trial that is likely to change the standard approach to treatment for these diseases.  His accomplishments are a perfect example of exactly what we wish to honor with the Taubman Prize.” 

The Taubman Prize was established to annually recognize the clinician-researcher who has done the most to transform laboratory discoveries into clinical applications for patients suffering from disease.

“Dr. Dietz exemplifies the passion and persistence of physician-scientists everywhere,” said Alfred Taubman, founder and chair of the A. Alfred Taubman Medical Research Institute. “We established the Taubman Prize to recognize and encourage these dedicated researchers, whose drive to find effective treatments and cures is fueled by their interaction with the patients suffering from life-altering diseases.

“In honoring Dr. Dietz, an eminent scientist whose landmark findings may improve quality of life for tens of thousands of people, I am confident that we have accomplished our goal.”

Eva Feldman, M.D., Ph.D., director of the Taubman Institute and the Russell N. DeJong Professor of Neurology at the U-M Medical School, said the nominees for the prize included many of the most talented and accomplished clinician-scientists at the world’s top research institutions.

“It’s a tribute to the extraordinary nature of Dr. Dietz’s work that our selection committee unanimously agreed to award him the Taubman Prize,” said Feldman. “The Taubman Prize is meant to reward translational research and to help remedy the long-standing disconnect between research in the laboratory and the introduction of new treatments for patients. Researchers like Dr. Dietz are truly scientists who create cures.”

Dietz, a professor of pediatric cardiology and genetics expert at the Johns Hopkins University School of Medicine and a Howard Hughes Medical Institute investigator, began his research career studying Marfan syndrome. This potentially fatal connective tissue disease enlarges the aorta, the largest blood vessel in the body and the main highway for blood as it leaves the heart. The syndrome makes the aorta more inclined to tear or burst, an often fatal event. About one in 5,000 people have Marfan syndrome, which is thought to have afflicted historical figures such as Julius Caesar, Abraham Lincoln and Charles de Gaulle. Olympic volleyball player Flo Hyman died in 1986 from an aneurysm that was the result of Marfan syndrome.

Working with his Johns Hopkins colleague, Bart Loeys, M.D., Ph.D., Dietz also was the first to observe and describe a related genetic disorder that bears their names, Loeys-Dietz syndrome.

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 2012 Taubman Prize.

Nominations for the 2013 Taubman Prize are being accepted now on the Taubman Institute website at www.taubmaninstitute.org . Self-nomination is permitted, and the prize is open to all clinician-scientists worldwide with the exception of University of Michigan researchers, who are eligible for other Taubman Institute funding. In keeping with the Taubman Institute mission to support high-risk, high-reward work by physician-scientists, clinical practice is an important component of the judging criteria, and all nominees must possess an M.D.

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