Prions (PDF)

Prion-related diseases, known as transmissible spongiform encephalopathies (TSEs), are infectious, fatal neurodegenerative disorders for which there is no cure, treatment, nor even a means for early diagnosis. The horrific advent of Mad Cow Disease -- transmitted to humans through eating meat from steers sickened by bovine spongiform encephalopathy --brought prion-related diseases international attention. Exceptionally dramatic, these diseases progressively and inexorably destroy the cognitive, motor, and sensorial skills that are the essence of human beings. Prions: The New Biology of Proteins provides a well-organized overview of what is known about prion-related diseases. This comprehensive work reviews the symptoms, epidemiology, and neuropathology of the disease. It focuses on evidence supporting the idea that TSEs result from a novel disease mechanism: transmission by replication of the misfolding of a single protein in the absence of nucleic acids. Following this hypothesis, the book examines the structure, conversion, and mechanism of prion propagation and details its cellular biology. It explores the transmission, discusses the challenges involved with diagnosis, and considers various therapeutic avenues that are presently being explored. A cohesive volume that integrates the pioneering work of many researchers, this book is authored by Claudio Soto, an internationally renowned researcher whose innovative work has led to an increased understanding of the heretical biology of prions and the development of novel strategies for treating and diagnosing neurodegenerative diseases. As protein misfolding diseases are his specialty, Soto also looks at the phenomenon from a wider perspective. He examines other diseases that display folding aberrations, considers how commonly such aberrations occur in nature, and asks readers to open their minds to consider the impact of prions on broader areas of biology, public health, and biotechnological strategies.