Stanley Prusiner

Origins and early life

Stanley Ben Prusiner was born in Des Moines, Iowa, on 28 May 1942, the son of Miriam Spigel and of Lawrence Prusiner, a navy officer during the war and later an architect, and he grew up between Des Moines and Cincinnati.

Work and career

His father's father had come to America from Moscow in 1896 as a young boy and grown up in Sioux City, Iowa, as did Stanley's father, among many other Russian Jews, and in 1997 a San Francisco Jewish weekly described him as a longtime member of the city's Congregation Sherith Israel. He studied chemistry and then medicine at the University of Pennsylvania, graduating in 1968, met his wife, Sandy Turk, a mathematics teacher, during his internship in San Francisco, and spent three years studying bacterial enzymes at the National Institutes of Health.

In 1972, as a neurology resident at the University of California, San Francisco, he admitted a woman dying of Creutzfeldt-Jakob disease, then blamed on a slow virus, and the nature of its agent became his life's work. Working on scrapie, a related disease of sheep, he found that his purified infectious preparations held protein but no nucleic acid, the genetic material of every known germ. In 1982 he proposed that the agent was a protein alone and named it the prion, from proteinaceous and infectious, an idea most virologists greeted with disbelief.

From 1984 his team and its partners showed that the prion gene exists in healthy animals and people, and that disease begins when the normal protein folds into a harmful shape that converts others in turn.

Recognition

The 1997 Nobel Prize in Physiology or Medicine was awarded to him alone, “for his discovery of Prions – a new biological principle of infection”. In 1999 he founded the Institute for Neurodegenerative Diseases in San Francisco, which studies misfolded proteins in Alzheimer's disease and related disorders, and in 2010 he received the National Medal of Science.