[Thelma]

http://news.ucsf.edu/releases/ucsfs-prusiner-receives-presidents-national-medal-of-science/

UCSF's Prusiner receives President's National Medal of Science
October 15th, 2010
UCSF Nobel laureate Stanley B. Prusiner, MD, UCSF professor of neurology and director of the Institute for Neurodegenerative Diseases, today (Oct. 15, 2010) was named to receive the National Medal of Science, the nation's highest honor for science and technology.
Prusiner was among 10 recipients named by President Barack Obama. In addition, three individuals and one team were named as recipients of the National Medal of Technology and Innovation. All of the awardees will receive their medals at a White House ceremony later this year.
The National Medal of Science honors individuals in a variety of fields for pioneering scientific research that has led to a better understanding of the world, as well as to the innovations and technologies that give the United States its global economic edge. It is administered by the National Science Foundation, established by Congress in 1959.
Prusiner received the medal for his discovery of and ongoing research on a novel infectious agent, which he named the prion (PREE-on). The prion, composed solely of protein, causes bovine spongiform encephalopathy, or "mad cow" disease, and other related fatal neurodegenerative diseases in animals and humans. Prusiner and colleagues are working on better methods for detecting prions and on the development of effective treatments for prion diseases.
In 1997, Prusiner won the Nobel Prize in Physiology or Medicine for his discovery.
In recent years, Prusiner has intensified his focus on developing preventions and cures for the neurodegenerative diseases that include the prion diseases as well as the more common disorders, Alzheimer's and Parkinson's diseases, at the Institute for Neurodegenerative Diseases (IND), involving faculty at six University of California campuses. He considers Alzheimer's disease one of the most devastating and underfunded illnesses affecting society, and is advocating strongly for a substantial increase in federal funding for research.
"Alzheimer's disease afflicts 5.3 million people in America," said Prusiner. "Each year, about 500,000 people die with cancer and about the same number die with Alzheimer's. Yet, Alzheimer's research receives only $450 million annually from the National Institutes of Health, about 1/15th that devoted to cancer research. We urgently need to increase funding to make substantial breakthroughs."
Pioneering Prion Research
Noting Prusiner's "vision, perseverance and courage in the face of skeptics as he pioneered prion research two decades ago," UCSF Chancellor Susan Desmond-Hellmann, MD, MPH, called him an "inspiration to us all."
"His revelations regarding prions have dramatically impacted scientists' understanding not only of prion diseases, but also of the more common neurodegenerative diseases, including Alzheimer's disease, Parkinson's disease and amyotrophic lateral sclerosis," she said. "He represents the best in translational science - driving basic research toward therapies for patients."
Prusiner's discovery that a protein alone could be infectious was considered heretical when he reported it in 1982. At the time, scientists believed that infections could only be transmitted by bacteria, viruses, fungi and parasites, all which have genomes composed of either DNA or RNA. Protein is composed of amino acids.
Prusiner determined that a normal form of prion protein exists in all mammals, including humans, and that the protein becomes lethal when it acquires an aberrant shape in brain cells. He showed that a particular segment of the normal prion protein loses its corkscrew-shape structure (known as an alpha helix) and flattens into so-called beta sheets. Once it does, the beta sheet form latches on to a neighboring prion protein, twisting its normal spiral tendrils flat. This activity occurs throughout the brain in a domino effect. The damaged proteins ultimately cause death of nerve cells in the brain.
His research has informed scientists' understanding that all neurodegenerative diseases - including Alzheimer's disease, frontotemporal dementias, Parkinson's disease, amyotrophic lateral sclerosis and Huntington's disease - involve the accumulation of proteins that have lost their normal shape. Each disease is caused by a different protein, but research on the abnormal prion protein has fueled scientists' understanding of a prion-like phenomenon in each of the diseases.
"The work of Dr. Prusiner has led to more knowledge about prion diseases than any other form of neurodegeneration," said Jeffrey Bluestone, PhD, UCSF executive vice chancellor and provost. "But, as importantly, these discoveries have forged a new understanding of neurodegenerative disease processes that will lead to new treatments for devastating diseases such as Alzheimer's and Parkinson's."
The awarding of the National Medal of Science "is a fitting recognition of Dr. Prusiner's visionary work, and a great honor for UCSF," said Sam Hawgood, MBBS, dean of the School of Medicine and vice chancellor of medical affairs. "He exemplifies UCSF's commitment to excel in biomedical research and bring discoveries to patients."
Advancing Neurosciences Research
Today, at age 68, Prusiner is a key force behind UCSF's development of a neuroscience building that will gather under one roof clinicians, clinician-researchers and basic scientists to accelerate advances against such disorders as Alzheimer's disease, Parkinson's disease, multiple sclerosis, stroke, migraine, epilepsy, autism, mental retardation and cerebral palsy. The building is a milestone in the evolution of UCSF's world-class neuroscience enterprise.
"This culminates a 10-year dream," said Prusiner. "This building will bring together some of the best scientists in the world to work on these common diseases of the brain. The opportunity for major progress is tremendous."
The research space provided by the neuroscience building and the space in the adjacent Arthur and Toni Rembe Rock Hall neuroscience building at UCSF Mission Bay will together constitute "more than 400,000 square feet dedicated to studying these extremely complex, challenging diseases," Prusiner said. "UCSF Mission Bay will be one of the biggest neuroscience complexes in the world."
The need for such intensive focus is crucial, he said. "The last three decades have seen unprecedented advances in our understanding of the molecular, genetic and cellular basis of neurodegenerative diseases. We have a fundamental understanding of the proteins underlying such devastating illnesses as Alzheimer's disease, Parkinson's disease, frontotemporal dementias, ALS and prion diseases, and are moving in on the development of targeted drug therapies.
"But we have strides yet to make. No effective treatments have been introduced for any neurodegenerative disease since L-dopa was introduced for Parkinson's disease in 1967. And even that drug, while effective in treating symptoms until the brain becomes resistant, does not stop the progression of the underlying neurodegeneration."
Prusiner is the fourth UCSF scientist to be awarded the National Medal of Science. Herbert Boyer, PhD, professor emeritus of biochemistry, received the award in 1990 for co-co-pioneering the development of recombinant DNA technology, and pioneering the use of the technology to create medically useful drugs. The seminal breakthrough led to the birth of biotechnology.
J. Michael Bishop, MD, a chancellor emeritus of UCSF and professor of microbiology and immunology, received the award in 2003 for his co-discovery of proto-oncogenes—normal genes that can be converted to cancer genes by genetic damage. Harold Varmus, MD, who shared the Nobel Prize in Physiology or Medicine with Bishop in 1989, received the medal in 2001. Their work eventually led to the recognition that all cancer probably arises from damage to normal genes, and provided new strategies for the detection and treatment of cancer.
Along with Prusiner, one other University of California scientist was named today as 2010 National Medal of Science laureate: Marye Anne Fox, of the University of California, San Diego.
Provided by University of California -- San Francisco


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[paula_w]

from the CDC

http://www.cdc.gov/ncidod/dvrd/prions/

About Prion Diseases


Prion diseases or transmissible spongiform encephalopathies (TSEs) are a family of rare progressive neurodegenerative disorders that affect both humans and animals. They are distinguished by long incubation periods, characteristic spongiform changes associated with neuronal loss, and a failure to induce inflammatory response.

The causative agent of TSEs is believed to be a prion. A prion is an abnormal, transmissible agent that is able to induce abnormal folding of normal cellular prion proteins in the brain, leading to brain damage and the characteristics signs and symptoms of the disease. Prion diseases are usually rapidly progressive and always fatal.

Related conditions: prion diseases

• Creutzfeldt-Jakob Disease
• Spongiform Encephalopathy
• New Variant Creutzfeldt-Jakob Disease
• Fatal Familial Insomnia
• Kuru

• Insomnia
• Alzheimer's Disease
• Dementia
• Parkinson's Disease
• Ataxia

NIH
http://www.ghr.nlm.nih.gov/condition/prion-disease