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Review Essays of Academic, Professional & Technical Books in the Humanities & Sciences


Prions by Claudio Soto (CRC) offers a well-organized overview of prion-related diseases. It reviews the symptoms, epidemiology, and neuropathology of the disease, and focuses on the idea that TSEs result from a novel mechanism involving transmission by replication of the misfolding of a single protein. The author, a renowned innovator in the area of neurodegenerative diseases, examines the structure, conversion, and mechanism of prion propagation and details its cellular biology. He also looks at other diseases that display folding aberrations, considers how common such aberrations are, and speculates on the impact of prions on broader areas of biology, public health, and biotechnological strategies.

Transmissible spongiform encephalopathies (TSEs), also known as prion related diseases, are a group of infectious, fatal neurodegenerative disorders for which there is no cure, treatment, or early diagnosis. TSEs are dramatic diseases that rapidly, progressively, and inexorably destroy the cognitive, motor, and sensorial skills that are the essence of human beings. At the molecular level, the disease is likely caused by the misfolding of the prion protein, which accumulates in the brain and produces neurodegeneration. Several unprecedented scientific findings, which have directly confronted popular dogmas in biology, have put prion research in the spotlight. The experimental evidence strongly supports an entirely novel disease mecha­nism, involving disease transmission by replication of the misfolding of a single protein in the absence of nucleic acids. The popularity of prion dis­eases is also due to the panic produced by the recent appearance of a new human disease (variant Creutzfeldt-Jakob disease) that is transmitted by eating meat contaminated by bovine spongiform encephalopathy (BSE), bet­ter known as mad cow disease. Because of insufficient information available regarding the incubation time and the actual level of exposure to the con­taminated material, it is impossible to make any well-founded prediction about the future of this nascent epidemic.

This book attempts to combine a detailed and up-to-date description of the state of the knowledge in the field with an intriguing but tempered speculation of the putative implications of the findings to our current under­standing of biology. During the last few years we have begun to perceive the broader implications of the heretically attractive prion concept of trans­mission of biological information by propagation of alternative protein fold­ing. This book may contribute to liberating the imagination of the reader to see the new scientific world opened by prions, which impact broader areas of biology, public health, and biotechnological strategies for therapy and diagnosis.

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