Month: February 2014

The 2014 Harrington Prize for Innovation in Medicine: Harry C. Dietz, MD

Posted on by John Hawley

Harrington Discovery Institute and the American Society for Clinical Investigation honor Johns Hopkins Pediatric Cardiologist and Geneticist Harry C. Dietz, MD

Harry Dietz

The inaugural Harrington Prize for Innovation in Medicine has been awarded to pediatric cardiologist and genetics researcher Harry Dietz, MD, of The Johns Hopkins University School of Medicine.

The Harrington Prize for Innovation in Medicine, established this year by the Harrington Discovery Institute at University Hospitals Case Medical Center and the American Society for Clinical Investigation (ASCI), is a $20,000 honorarium that recognizes a physician-scientist who has successfully navigated the path to advance discovery into clinical application.

Dr. Dietz became a scientist as well as a pediatrician to better care for young patients with Marfan syndrome, a rare and potentially fatal connective tissue disease that enlarges the aorta, leading it to tear or burst. Dr. Dietz’s work provided new insights into the origins of the disease, which motivated clinical trials under his direction with commonly available medication. Children with Marfan syndrome have thus been offered new hope for longer and healthier lives. Dr. Dietz has also inspired research that may lead to a blood test to detect aortas at risk for rupture so that surgery can be timed appropriately.

“The extraordinary dedication and creative thought of Dr. Dietz has led to groundbreaking progress in understanding aortic aneurysms and connective tissue disorders,” said Peter Tontonoz, MD, PhD, the 2013 – 2014 President of the ASCI. “He and his team took basic genetic science and found a promising therapeutic agent – a commonly prescribed blood-pressure medication – that prevents aortic aneurysms from forming that will improve the quality of life and save thousands of lives each year.”

A committee composed of members of the ASCI Council and the Harrington Discovery Institute Scientific Advisory Board reviewed more than 60 nominations from eight countries before selecting Dr. Dietz as the recipient. This year’s award acknowledges Dr. Dietz’s “bedside to bench and back” efforts that have led to fundamental new insights into the biology of Marfan syndrome and related disorders, and the development of therapies to improve care standards.

“We are so pleased to join with the ASCI to honor Harry and his team’s remarkable work that has changed the way people think about Marfan syndrome as well as the quality of life of young children with the disease,” said Jonathan Stamler, MD, Director of the Harrington Discovery Institute and the Robert S. and Sylvia K. Reitman Distinguished Chair in Cardiovascular Innovation at University Hospitals and Case Western Reserve University School of Medicine. “Harry has made a difference and serves as a role model for all of us hoping to see our discoveries advanced into medicines that impact the lives of our patients.”

In addition to a $20,000 prize honorarium, Dr. Dietz will deliver the Harrington Prize Lecture at the 2014 ASCI and Association of American Physicians joint meeting on April 26 and publish a review in the April issue of the Journal of Clinical Investigation.

Dr. Dietz is the Victor A. McKusick Professor of Genetics in the Departments of Medicine, Pediatrics, and Molecular Biology and Genetics at The Johns Hopkins University School of Medicine and Director of the William S. Smilow Center for Marfan Syndrome Research.

The Harrington Prize, presented by the American Society for Clinical Investigation and the Harrington Discovery Institute at University Hospitals Case Medical Center, honors a physician-scientist who has moved science forward with achievements notable for innovation, creativity and potential for clinical application.

The Harrington Discovery Institute at University Hospitals Case Medical Center, part of a national initiative unveiled in February 2012 called The Harrington Project, is a nonprofit medical institute dedicated to physician-scientists, enabling them to transform breakthrough insights into novel therapies that enhance patient care. The Harrington Project is fueled by $250 million in donations and other funding, including $50 million from the Harrington Family.
Learn more about The Harrington Project.

The 2014 Stanley J. Korsmeyer Award: Beth Levine, MD

Posted on by John Hawley

Beth Levine

Photo credit: Brian Coats for UT Southwestern Medical Center.

Beth Levine, MD, Howard Hughes Medical Institute investigator, professor, and director of the Center for Autophagy Research at UT Southwestern Medical Center (UTSW), is the recipient of the 2014 American Society for Clinical Investigation’s Stanley J. Korsmeyer Award. The award recognizes Dr. Levine’s fundamental contributions to our understanding of autophagy — literally, “self-eating” — a housecleaning process in which cells destroy damaged proteins and organelles.

Inspired by Stanley Korsmeyer’s co-discovery of Bcl-2 as a B cell lymphoma oncogene, Dr. Levine began her independent research career by searching for proteins that interacted with Bcl-2. These experiments led to the identification of a gene she termed Bcl-2 interacting protein, or beclin 1, and her subsequent characterization of beclin 1 opened the molecular era of disease-related autophagy research. Dr. Levine showed that beclin 1 is an essential mammalian autophagy gene and important for preventing many tumors. One copy of the gene is lost in about of half of human breast and ovarian cancers; beclin 1 prevents lung cancer, liver cancers, and B cell lymphomas in mice; and Bcl-2 and other oncogenes inactivate beclin 1. Dr. Levine demonstrated how Akt, a gene in the insulin-signaling pathway that is activated in many cancers, inhibits autophagy by inactivating beclin 1, allowing unregulated tumor cell growth. More recently, her laboratory showed that the epidermal growth factor receptor — which is expressed at abnormally high levels by many types of cancer cells — deactivates autophagy by binding beclin 1, leading to increased rates of tumor growth and chemotherapy resistance in non-small cell lung carcinomas.

Dr. Levine’s work has also revealed the crucial role of autophagy in defense against intracellular pathogens. Her group showed that autophagy genes protect against lethal alphavirus encephalitis and Salmonella typhimurium infection, and found that a herpes simplex virus neurovirulence factor acts by antagonizing beclin 1. Furthermore, her work suggests that beclin 1 and the autophagy pathway slow the progress of neurodegenerative diseases, increase lifespan, and underlie the beneficial effects of exercise on glucose metabolism.

Recently, Dr. Levine and her colleagues identified an autophagy-inducing peptide, called Tat-beclin 1. Mice treated with this peptide are resistant to several infectious diseases. In additional experiments, the team demonstrated that human cells treated with Tat-beclin 1 are resistant to HIV infection and are more efficient at clearing mutant huntingtin protein aggregates. The peptide may thus have therapeutic potential in the prevention and treatment of a broad range of human diseases. Dr. Levine’s current research focuses on the role of autophagy in normal development and aging, the mechanisms by which autophagy genes suppress tumors, biochemical mechanisms that regulate beclin 1, and the role of autophagy in infection and exercise physiology.

Dr. Levine received her M.D. from Cornell University, completed a residency at Mount Sinai Hospital, and did postdoctoral training in infectious diseases and the neurobiology of viral pathogens at Johns Hopkins University. She was director of Virology Research at Columbia University from 1994 to 2004, and thereafter joined the UT Southwestern faculty. She holds the Charles Cameron Sprague Distinguished Chair in Biomedical Science. Dr. Levine was elected to the American Society for Clinical Investigation in 2000, the Association of American Physicians in 2006, and the National Academy of Sciences in 2013.

Related

Beth Levine receives the 2014 ASCI/Stanley J. Korsmeyer Award in the Journal of Clinical Investigation