American wins Nobel for discovery of infectious particles Prions termed 'new genre of disease-causing agents'


The Nobel Prize in physiology or medicine was awarded yesterday to Dr. Stanley B. Prusiner, a 55-year-old maverick scientist in San Francisco whose discovery of infectious particles called prions have been criticized by other researchers as unproved.

The Nobel committee compounded its departure from the tradition of rubber stamping well-accepted scientific work by awarding the prize to only one researcher, emphasizing its confidence in Prusiner's discovery of a "new genre of disease-causing agents."

These agents, neither bacteria nor fungi nor viruses, are proteins and have been linked to "mad cow" disease and other lethal brain-wasting conditions.

The Nobel committee said yesterday that Prusiner's discovery might eventually shed light on Alzheimer's disease and provided a foundation for new types of drug and other therapies for Alzheimer's and other demential ailments.

Most of Prusiner's critics do accept that prions exist, but not that they are necessarily agents of disease.

The committee cited Prusiner for discovering the rogue prion proteins as "a new biological principle of infection" and adding them "to the list of well-known infectious agents."

But some scientists doubt that they can cause disease because unlike other infectious agents, they contain no genetic material.

According to Prusiner's findings, harmless forms of prions exist as proteins in the brain. But prions can fold in an aberrant shape to become rogue, disease-causing agents. In time, the rogue prions, which are smaller than viruses, ravage the brain, killing brain cells, forming holes and turning it into something resembling a sponge.

Symptoms such as memory loss, loss of muscle control and insomnia vary, depending on which regions of the brain are damaged.

Prion diseases can be inherited, occur spontaneously or be transmitted in other ways. Scientists are investigating whether Britons affected by mad cow disease might have become ill from eating tainted beef.

For unknown reasons, the immune system does not defend against prions.

Prusiner has said that he began his research on prions in 1972 after one of his patients died from dementia resulting from Creutzfeldt-Jakob disease and he became determined to figure out what caused the condition.

Yesterday, he said he did not believe he was necessarily vindicated by the Nobel award. "Concepts are vindicated by the constant accrual of data and independent verification of data," he said. "No prize, not even a Nobel Prize, can make something true that is not true."

The Nobel Prize usually is shared by two or three people and generally is given long after controversies have been resolved. Prusiner is the first single winner since 1987 and only the sixth in the past 40 years.

Prusiner was born in Des Moines, Iowa, and earned his undergraduate and medical degrees at the University of Pennsylvania. He has spent his entire professional career at the University of California at San Francisco.

Pub Date: 10/07/97

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