Harry Baker and Rosalind Ridley have done an admirable job in assem bling this collection of articles that describe the methodology frequently used to study a group of CNS illnesses often referred to as the "prion diseases." Research on prions and the disorders that they cause has progressed relatively rapidly over the last decade since the discovery of the prion protein (PrP) that allowed the application of modem molecular biological and genetic tools. The power of these techniques is awesome and their use in deciphering the once mysterious prion diseases has brought a wealth of new...

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-Straussler-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...

Diseases such as Creutzfeldt-Jakob disease and kuru develop when PrP proteins form prions by misfolding, clumping together, and spreading from cell to cell. Over the past decade, a number of proteins have been reported to possess the characteristics of PrP prions. A growing collection of disorders has been found to be caused by prions; some of these prion diseases include such devastating illnesses as Alzheimer's and Parkinson's and possibly type 2 diabetes.

Written and edited by experts in the field, this collection from Cold Spring Harbor Perspectives in Medicine covers the...

Prions are best known as the agents of mad cow and related diseases, and a growing number of proteins with prion-like properties have been implicated in diseases such as Parkinson's. But some recently discovered prion-like proteins do not appear to induce pathological changes and, in fact, may be key players in basic biological processes such as transcription, immune regulation, and memory formation.

Written and edited by experts in the field, this collection from Cold Spring Harbor Perspectives in Biology examines the expanding roles of prions and prion-like proteins in health...