Dr. Stanley Prusiner, University of California San Francisco

The Brockman Medical Research Foundation has provided support for research being conducted by neurologist, Dr. Stanley Prusiner and his research team at the University of California San Francisco (UCSF).

Dr. Prusiner is Professor of Neurology and Biochemistry at UCSF and Director of the Institute for Neurodegenerative Diseases in San Francisco. He is also President-elect of the American Neurological Association.

The editor of 11 books and author of more than 500 research articles, Dr. Prusiner’s contributions to scientific research and medicine are internationally recognized.

Among Dr. Prusiner’s many prizes are the Albert Lasker Award for Basic Medical Research (1994) and the Nobel Prize in Physiology or Medicine in 1997 for his discovery of Prions and his descriptions of their roles in the pathogenesis and pathophysiology of certain severely disabling neurodegenerative disorders including Creutzfeldt-Jacob disease in humans and bovine spongiform encephalopathy (mad cow’s disease) in cattle and transferrable to humans.

Dr. Prusiner and his research colleagues have since studied intensively the role of Prions in common, severely-disabling neurodegenerative diseases in humans, including Alzheimer’s Disease, Parkinson’s Disease, many of the frontotemporal dementias, as well as some forms of Amyotrophic Lateral Sclerosis (ALS).

Prions are composed of host-encoded proteins that adopt alternative conformations, which are self-propagating. As Prions accumulate, they begin to manifest toxicity and eventually reach and cross a threshold that is destructive and deadly to neurons.

At present, there are no effective medications to treat the underlying causes of Alzheimer’s, Parkinson’s or related neurodegenerative disorders. Although the signs and symptoms of some of these neurodegenerative diseases can be treated with drugs that lessen the severity of signs and symptoms, improvements generally are temporary.

The grant by the Brockman Medical Research Foundation supports research toward the development and discovery of medications that inhibit the accumulation of misfolded proteins and, thereby, advance therapeutics for certain common neurodegenerative diseases.

Such a discovery would have a profound positive impact on public health in the United States, and worldwide. For example, approximately 1.5 million Americans are afflicted with Parkinson’s disease, while about 5.5 million Americans suffer with Alzheimer’s disease. Approximately 500,000 people die annually in the United States with Alzheimer’s, while another 500,000 new cases of Alzheimer’s are diagnosed.

The number of Alzheimer’s disease and Parkinson’s disease cases is expected to increase dramatically as the longevity of populations worldwide continues to rise. By 2050, for example, it is estimated that 14 million Americans will have Alzheimer’s disease, unless effective new interventions and preventative measures are discovered.

Project Highlights

  • Project Focus: Research toward the development and discovery of medications that inhibit the accumulation of misfolded proteins