Prions: The New Biology of Proteins PDF
Preview Prions: The New Biology of Proteins
PRIONS The New Biology of Proteins PRIONS The New Biology of Proteins CLAUDIO SOTO Boca Raton London New York A CRC title, part of the Taylor & Francis imprint, a member of the Taylor & Francis Group, the academic division of T&F Informa plc. Cover illustration drawn by Eric D. Smith, Harvard University. CRC Press Taylor & Francis Group 6000 Broken Sound Parkway NW, Suite 300 Boca Raton, FL 33487-2742 © 2006 by Taylor & Francis Group, LLC CRC Press is an imprint of Taylor & Francis Group, an Informa business No claim to original U.S. Government works Version Date: 20130919 International Standard Book Number-13: 978-1-4200-4012-8 (eBook - PDF) This book contains information obtained from authentic and highly regarded sources. Reasonable efforts have been made to publish reliable data and information, but the author and publisher cannot assume responsibility for the validity of all materials or the consequences of their use. 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CCC is a not-for-profit organization that provides licenses and registration for a variety of users. For organizations that have been granted a photocopy license by the CCC, a separate system of payment has been arranged. Trademark Notice: Product or corporate names may be trademarks or registered trademarks, and are used only for identification and explanation without intent to infringe. Visit the Taylor & Francis Web site at http://www.taylorandfrancis.com and the CRC Press Web site at http://www.crcpress.com Dedication To Claudia, Andres, Kevin, Dylan, and my little girl to be.… Preface 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 mecha- nism, involving disease transmission by replication of the misfolding of a single protein in the absence of nucleic acids. The popularity of prion dis- eases 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), bet- ter known as mad cow disease. Because of insufficient information available regarding the incubation time and the actual level of exposure to the con- taminated 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 under- standing of biology. During the last few years we have begun to perceive the broader implications of the heretically attractive prion concept of trans- mission of biological information by propagation of alternative protein fold- ing. I hope that this book can 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. Although I regretted it many times during the long process of preparing this book, I decided to write it entirely myself in order to give continuity to the text, maintain a homogenous style, and provide an appropriate connec- tion between chapters. The decision was also guided by a desire to avoid important gaps and repetitions, which are frequent in multiauthored books. I could not have completed this book without the constant support of my family, lab members, and close collaborators. To them I extend my sincere gratitude for their patience, help, and illuminating discussions. Author Claudio Soto is currently the Charlotte-Warmoth professor of neurology, human biological chemistry and genetics, and neuroscience and cell biology, and director of the Protein Misfolding Disorders Research Unit at the Uni- versity of Texas Medical Branch in Galveston. Dr. Soto was recently awarded the Green Distinguished University Chair in Neurosciences and was appointed Director of the new George and Cynthia Mitchell Center for Alzheimer’s Disease Research. He received his Ph.D. in biochemistry and molecular biology from the University of Chile in 1992 and was a postdoc- toral fellow at the Catholic University of Chile and at the New York Univer- sity School of Medicine, where he became an assistant professor of research in 1995. Between 1999 and 2003, Dr. Soto was senior scientist, head of the Department of Molecular Neuropathology, and scientific advisor for neuro- biology at Serono International in Switzerland. For the past 12 years, he and his colleagues have engaged in research into the molecular basis of neuro- degenerative diseases associated with the misfolding of proteins and their accumulation in the brain, focusing particularly on Alzheimer’s and prion- related disorders. His work has led to the development of novel strategies for treatment and diagnosis of neurodegenerative diseases. Dr. Soto has published more than 80 scientific articles in some of the most prestigious peer-reviewed journals. He has received numerous awards and has been invited to give presentations at more than 50 international meetings worldwide. Dr. Soto is a member of the editorial board of several scientific journals and has acted as adviser and reviewer for many institu- tions, including the World Health Organization, the U.S. National Institutes of Health, and the European Commission, among others.
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