Stanley Prusiner, M.D., a neurobiologist at the University of California at San Francisco, was awarded the 1997 Nobel Prize in Medicine for his groundbreaking discovery and definition of a new class of disease-causing agents called prions (pronounced pree-ons). The Nobel Prize, is the most prestigious award given for research in medicine.
Dr. Prusiner's award is the culmination of 25 years of sometimes controversial research on the prion, a natural human protein that, under certain conditions, can interact with other prion proteins, ultimately forming harmful deposits in the brain. The American Health Assistance Foundation (AHAF) has awarded more than $1.2 million in research grants through its Alzheimer's Disease Research program to Dr. Prusiner to develop his prion theory as a model for Alzheimer's disease.
Prions have been implicated in dementia-causing diseases such as mad cow disease and scrapie in animals, and Creutzfeldt-Jakob Disease (CJD) and Gerstmann-Straussler-Scheinker syndrome (GSS) in humans. Unlike infectious agents such as bacteria, viruses and parasites, whose ability to grow and reproduce is governed by genetic material made up of RNA and DNA, prions appear to be made up entirely of proteins with no accompanying DNA or RNA. Prions are present in normal cells, and the gene that codes for the production of the prion protein is part of a normal human chromosome.
Since 1985, the American Health Assistance Foundation has supported studies of the structures and properties of prions, and investigations that led to the purification and identification of the prion protein in the brains of scrapie-infected sheep. AHAF also awarded a grant to Dr. Prusiner to study CJD and GSS, using molecular biology methods to introduce genes from mutated prion proteins into mice to create an animal model for these diseases. His AHAF grant focused on the development of a new system to determine when in the life of a mouse the prion protein leads to disease. He is also studying a method to prevent prion disease by blocking prions from converting normal proteins into more prions.
There are similarities between the loss of brain function in prion diseases and in Alzheimer's disease, and an understanding of how prion diseases begin and develop will add to our understanding of what happens to the brain in Alzheimer's disease. Dr. Prusiner's research may one day lead to a treatment and a cure for Alzheimer's.
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