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基本説明
Based on the 1st International Workshop on Conformational Diseases.
Full Description
'Conformational diseases' are a group of heterologous disorders in which a constituent host protein undergoes a change in conformation or fluctuation in shape with resultant self-association and deposition. The etiology of many diseases has now been traced to proteins that are either misfolded from the start or have undergone post-translational conformational alterations. The articles in this compendium reflect the recent advances and exponential growth of the field. They also provoke many questions. How do malfolded proteins cause disease? How are such diseases transmitted from one individual to another, or from animals to humans? And how can one prevent these conformational alterations or combat their deleterious effects? These and many other questions remain to be answered before we can achieve a clearer picture of conformational disorders. The beautifully produced compilation is divided into seven sections: Clinical aspects, theoretical considerations, experimental studies, models in vivo and in vitro, genetics, transgenetics and cell biology, prospects for treatment, and further considerations. Contributions include original articles by leading scientists, including Nobel laureate G. Carleton Gajdusek, Charles Weissmann, Shuguang Zhang, Michael Altman and Alexander Rich, Michel Goldberg, F.J. Stevens, M. Schiffer and Y. Argon. These are complemented by seminal papers that have already appeared in top-ranking journals, written by outstanding scholars such as Nobel laureate Stanley Prusiner, philosopher Henri Atlan, Christopher Dobson, Reed Wickner, Jean Sipe and many others. The resulting compilation is a comprehensive handbook on conformational diseases that encompasses many of the central elements in the field.
Contents
The structural basis of protein folding and its links with human disease; molecular recognition in vivo and in silico; protein misfolding and prion diseases; molecular genetics of transmissible spongiform encephalopathies - an introduction; on the cell biology of mammalian prions - do prions replicate on "rafts"?; the message of ubiquitin immuniohistochemistry in conformational neurodegenerative diseases; fantasy of a "virus" from the inorganic world - molecular casting and atomic ghost replicas - anyloid enhancind factors are scrapie-like agents; epigenesis and self-organization - new perspectives in biology and medicine; Nobel speece - prions.
