[Congressional Record Volume 143, Number 140 (Thursday, October 9, 1997)]
[Extensions of Remarks]
[Page E1987]
From the Congressional Record Online through the Government Publishing Office [www.gpo.gov]


  TRIBUTE TO DR. STANLEY B. PRUSINER, A ``MOZART OF SCIENCE,'' ON HIS 
                 RECEIVING THE NOBEL PRIZE IN MEDICINE

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                            HON. TOM LANTOS

                             of california

                    in the house of representatives

                       Thursday, October 9, 1997

  Mr. LANTOS. Mr. Speaker, it is my privilege and pleasure to hail the 
accomplishments of Dr. Stanley B. Prusiner, the 1997 recipient of the 
Nobel Prize in Medicine. Dr. Prusiner, a professor at the University of 
California San Francisco, joins 30 other Nobel laureates in the UC 
system, including UCSF's two previous medical honorees--microbiologists 
J. Michael Bishop and Harold Varmus, the current head of the National 
Institutes of Health. Dr. Prusiner was awarded this premier distinction 
for his landmark discovery of prions, rogue protein particles that 
function as infectious agents. This remarkable innovation could 
eventually lead to a cure for dreaded neurological diseases such as 
Alzheimer's, Parkinson's, Huntington's, and amyotropic lateral 
sclerosis, ALS, better know as Lou Gehrig's disease. In the citation 
announcing Prusiner's $1 million prize, Sweden's noted Karolinska 
Institute lauded the social impact of his achievement.
  ``Stanley Prusiner's discovery provides important insights that may 
furnish the basis to understand the biological mechanisms underlying 
other types of dementia-related diseases, for example Alzhemier's 
disease, and establishes a foundation for drug development and new 
types of medical treatment strategies.''
  For Dr. Prusiner and for his entire research team at UCSF, this 
recognition marks the zenith of a 15-year battle for a revolutionary 
theory that flew in the face of earlier scientific judgments about the 
causes of communicable brain diseases.
  Prusiner's commitment to using his medical genius to helping others 
began long before his discovery of prions. Born in Des Moines, IA, he 
graduated from the University of Pennsylvania Medical School and, after 
long doing biochemistry research at the National Institutes of Health, 
moved to the Bay Area in 1972 to begin his residency in neurology at 
UCSF. That year, a pivotal event shaped the direction of Prusiner's 
expertise: He began treating a Marin County woman affected with 
Creutzfeldt-Jakob disease, an exceptionally rare and always fatal 
condition that mercilessly destroys the brain. Prusiner's patient 
passed away after 7 weeks in the hospital, but her sickness impelled 
her doctor to examine further links between Creutzfeldt-Jakob and 
similar neurological illnesses, and to seek the cause of these 
devastating diseases. ``At that time,'' said Prusiner years later, 
``most people believed that the brain diseases were caused by slow 
viruses, but since I didn't know any virology, I figured I ought to 
look for some other explanation--and that's when I started hunting for 
proteins that might be involved.'' This research continued throughout 
his tenure as a Howard Hughes Investigator at UCSF from 1976 to 1981, 
culminating in his development of the prion theory in 1982.
  Prusiner's then-radical pronouncement stated that the cause of 
Creutzfeldt-Jacob and related maladies was not a virus at all; rather, 
these illnesses emanate from prions, biologically unique proteins which 
contain no DNA. Rather, in place of genetic reproduction, prions 
convert neighboring proteins, creating more disease-causing agents. 
This phenomenon has a devastating effect on nerve cells in the brain, 
ravaging tissue and leading to a certain death.
  The scientific community greeted the prion theory with disbelief and 
outright criticism that targeted not only Prusiner's conclusions, but 
his ethics as well. His financial grants quickly vanished, and he was 
forced to operate for years with only in-house grants from the loyal 
UCSF administration. These frustrations strengthened Prusiner's 
dedication to his work and as the years progressed, the case for the 
prion theory became stronger and stronger. His opponents found little 
evidence to discredit his conclusions, and Prusiner and his dedicated 
team of researchers, notably brain pathologist Stephen DeArmond and 
pharmacologist Stephen Cohen, published hundreds of papers 
substantiating the role of the prion in a variety of contagious 
neurological diseases.
  The grants returned, with significant contributions including a $2.5 
million prize from the W.M. Keck Foundation in Los Angeles and the 
Israeli Government's prestigious $100,000 Wolf prize. Such resources 
enabled Prusiner to tie the existence of prions to the recent British 
epidemic of bovine spongiform encepalopathy, BSE, better known as mad 
cow disease, and to chart the course for eventual cures to BSE and 
other disorders. He also won the coveted Albert Lasker Basic Medical 
Research Award in 1994, generally regarded as a strong indicator of a 
future Nobel Prize. In the words of his colleagues, neurologist and 
biochemist Jiri G. Safar, Prusiner ``carried on his shoulders the 
burden of proving this extraordinary new idea. * * * He single-handedly 
validated his theory. To do that, it takes a person of strong 
conviction and real guts.'' Mr. Speaker, we are all in debt to the 
courage of this outstanding man.
  Dr. Prusiner's next challenge is to eradicate these diseases from the 
face of this planet. In the aftermath of the BSE outbreak in Great 
Britain, he has used his findings concerning the replicating and 
infectious nature of prions to lobby the Food and Drug Administration 
and the Department of Agriculture to protect our food supply and make 
sure that such a plague will never occur in this country. Prusiner's 
research will also continue to seek the causes of Alzheimer's, 
Parkinson's, Huntington's, and ALS. Once the origins of these diseases 
are discovered, treatments such as gene therapy and prion-blocking 
medications may be created to cure them or to prevent them from 
spreading. Prusiner expects that within the next 5 to 10 years we will 
see a drug to stop the progression of Creutzfeldt-Jakob, the disease 
that led him to this area of neurological research a quarter century 
ago.
  Mr. Speaker, Dr. Prusiner has earned our utmost gratitude and 
respect. As his UCSF colleague Dr. DeArmond remarked, he is truly a 
``Mozart of science.'' Prusiner's brilliance, dedication, and, most of 
all, his persistence are a credit to his country and to the San 
Francisco community where he has lived with his wife, Sandy, and his 
family for over 25 years. I ask all of my colleagues to join me in 
congratulating the 1997 recipient of the Nobel Prize in Medicine, Dr. 
Stanley B. Prusiner, for his most-deserved award, for his devotion to 
finding answers to questions that have vexed the scientific world for 
generations, and for his dogged commitment to standing up for his life-
saving beliefs in the face of cynicism and skepticism.

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