Columbia University will award the 1997 Louisa Gross Horwitz Prize to Dr. Stanley B. Prusiner, professor of neurology and biochemistry of the University of California, San Francisco on Thursday October 23. Dr. Prusiner, winner of this year’s Nobel Prize in Medicine, is honored for his ground breaking work on prions, a protein capable of transmitting degenerative neurological diseases. The Louisa Gross Horwitz Prize, established in 1967, is given annually for outstanding research in biology or biochemistry. Research previously recognized by other awards, such as the Nobel Prize, is not eligible. The Horwitz Prize committee selected Dr. Prusiner for the award in May. For several decades, scientists have believed that nucleic acids–either DNA or RNA–were required for the development of infection. But in 1982, Dr. Prusiner stunned the scientific community by proposing that the infectious agent in scrapie–a neurodegenerative disease of sheep–was not a virus or bacteria, but a new agent containing only protein, which he called a prion. Prions–for “proteinaceous infectious particles”–are a misshapen form of a usually harmless protein that exists in the brain. Prions are thought to cause disease by forcing normal protein to also change shape, thereby becoming toxic to brain cells. They have been linked to certain neurodegenerative disorders, such as bovine spongioform encephalitis (better known as “mad cow disease”) and Creutzfeldt-Jakob disease, which is similar to mad cow disease but occurs in humans. In these diseases, holes develop in the brain, resulting in dementia and eventually death. In England, more than 130,000 cows have been affected by mad cow disease since the 1980s. Scientists believe the cows were infected by a food supplement made of the meat and bone meal from sheep that had scrapie. Researchers are now investigating whether people who ate the contaminated beef subsequently developed mad cow disease. Other diseases in the same class include fatal familial insomnia, Gertsmann-Straussler-Scheinker disease, and Kuru. Dr. Prusiner’s work on prions has generated much controversy. “His findings go against the central dogma of biology, which is that you can transmit information only with DNA and RNA,” says Dr. Michael Shelanski, the Francis E. Delafield Professor and Chairman of the department of pathology at Columbia-Presbyterian Medical Center. “But in chemistry we know that there can be some passing of information based on structure.” Prions can be seen as one end of a spectrum of disease-causing agents, he says. “After all, viruses don’t `live’ by themselves. They take command of the cell machinery and use that cell’s genes to reproduce. Prions take this a step further and dispense with the nucleic acid. So you could view prions as the ultimate reduction.” “At many junctures, the criticisms of both Dr. Prusiner and his work were severe,” says David I. Hirsh, the Robert Wood Johnson, Jr. Professor and Chairman of the department of biochemistry and molecular biophysics at CPMC and chairman of the Horwitz Prize committee.
“Nonetheless, Dr. Prusiner persisted and has almost single-handedly constructed the arguments that have led to widespread acceptance of his work.” Columbia President George Rupp will present the Horwitz prize in ceremonies October 23. That same day at 2 p.m. in the Alumni Auditorium at CPMC, Dr. Prusiner will speak on “Prions–Fatal Conformational Changes in Humans and Cows.” The Louisa Gross Horwitz Prize was established under the will of the late S. Gross Horwitz in memory of his mother. She was the daughter of Samuel David Gross (1805-1889), a prominent Philadelphia surgeon who pioneered methods for suturing nerves and tendons, and later served as president of the American Medical Association. Since the Horwitz Prize was first presented in 1967, more than half of its recipients have gone on to win the Nobel Prize in Physiology or Medicine, or in Chemistry. Most recently, Edward B. Lewis and Christiane Nusslein-Volhard, who shared the 1995 Nobel in Physiology, won the Horwitz in 1992.