Stanley B. Prusiner – författare

In this “utterly fascinating” science memoir, the Nobel Prize–winning author chronicles his revolutionary discovery of a major cause of brain diseases (The New York Times).   In 1997, Stanley B. Prusiner received the Nobel Prize in Physiology or Medicine for his research on devastating brain diseases. That he was the award’s sole recipient was entirely appropriate. His struggle to identify the agent responsible for scrapie and mad cow disease in animals, and Creutzfeldt-Jakob disease in humans, had been waged largely alone, and in some cases, in the face of strenuous opposition.   In Madness and Memory, Prusiner recounts the journey to his remarkable discovery of prions—infectious proteins that replicate and cause disease, but surprisingly contain no genetic material. Along the way, he sheds light on the world of contemporary science and the meticulousness and perseverance it requires. Through his telling, the agony and triumph of years of research comes to life, along with fascinating portraits of fellow scientists racing to make breakthrough discoveries.   Prusiner’s hypothesis, once considered heresy, now stands as accepted science and provides a path toward conquering such pervasive scourges as Alzheimer’s, Parkinson’s, Lou Gehrig’s, and others diseases.   “Madness and Memory is the story of one of the most important discoveries in recent medical history, and it is also a vivid and compelling portrait of a life in science.” —Oliver Sacks, MD, neurologist and author of Hallucinations

Slow Transmissible Diseases of the Nervous System

Le neurobiologiste américain Stanley B. Prusiner a obtenu le prix Nobel pour sa découverte des prions. Il a vécu une aventure scientifique peu commune. D’abord fasciné par la maladie de Creutzfeldt-Jakob, forme de démence due à une dégénérescence du cerveau, il se voit confronté, au début des années 1990, à la célèbre maladie de la vache folle, qui produit des effets comparables chez les bovins. Pour expliquer cette encéphalite spongiforme dont on pense qu’elle va causer une gigantesque épidémie, le monde de la recherche se lance sur la piste d’un agent infectieux classique — bactérie, parasite ou virus. Prusiner est persuadé qu’il s’agit d’un agent biologique entièrement nouveau, une « protéine infectieuse », ou prion. Les protéines étant les constituants ordinaires des êtres vivants, l’idée même du prion est une hérésie très mal accueillie par le monde de la biologie. Elle se révèle pourtant justifiée, au terme d’années de lutte et de controverses très instructives sur le fonctionnement du milieu scientifique. Les prions, protéines déformées, sont bien responsables des encéphalites, et ils jouent aussi un rôle majeur dans les maladies de Parkinson et d’Alzheimer. Leur découverte est une aventure scientifique haletante, hors des sentiers battus. « Ce livre relate une des plus importantes découvertes de l’histoire récente de la médecine ; c’est aussi un portrait sur le vif d’un grand scientifique dont le courage et la ténacité ont été mis à rude épreuve face au scepticisme général provoqué par son hypothèse des prions. » Oliver Sacks. Stanley B. Prusiner a obtenu le prix Nobel de médecine en 1997. Il est le directeur de l’Institut des maladies dégénératives de l’Université de Californie à San Francisco.

A series of remarkable discoveries in the past three decades have led to the molecular and genetic characterization of the transmissible pathogen causing scrapie in animals and a quartet of human illnesses: kuru, Jakob-Creutzfeld disease, Gerstmann-Sträussler-Scheinker disease, and fatal familial insomnia. To distinguish this pathogen from viruses and viroids, the term "prion" was introduced to emphasize its proteinaceous and infectious nature.Stanley B. Prusiner, editor of this volume, was awarded the 1997 Nobel Prize in Physiology and Medicine for his pioneering discovery of prions. The book reviews advances in studies of prions, which - as considereable evidence indicates - are novel pathogens composed only of protein.

A series of remarkable discoveries in the past three decades have led to the molecular and genetic characterization of the transmissible pathogen causing scrapie in animals and a quartet of human illnesses: kuru, Jakob-Creutzfeld disease, Gerstmann-Sträussler-Scheinker disease, and fatal familial insomnia. To distinguish this pathogen from viruses and viroids, the term "prion" was introduced to emphasize its proteinaceous and infectious nature.Stanley B. Prusiner, editor of this volume, was awarded the 1997 Nobel Prize in Physiology and Medicine for his pioneering discovery of prions. The book reviews advances in studies of prions, which - as considereable evidence indicates - are novel pathogens composed only of protein.