ENGLISH

Prions Prions Prions

By S. B. Prusiner (auth.) , Stanley B. Prusiner M.D. (eds.)

Book information

Publisher
Springer-Verlag Berlin Heidelberg
Year
1996
ISBN
9783642646409, 9783642609831
Language
english
Format
PDF
Filesize
7 MB (7040973 bytes)
Series
Current Topics in Microbiology and Immunology 207
Edition
1
Pages
163\166
Time added
2020-08-30 06:11:09
Virology; Neurology

Description

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-Sträussler-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 book reviews advances in studies of prions, which - as considereable evidence indicates - are novel pathogens composed only of protein. Front Matter....Pages i-vii Human Prion Diseases and Neurodegeneration....Pages 1-17 Fatal Familial Insomnia and Familial Creutzfeldt-Jakob Disease: A Tale of Two Diseases with the Same Genetic Mutation....Pages 19-25 Human Prion Disease and Human Prion Protein Disease....Pages 27-34 Prion Strains....Pages 35-47 Structures of Prion Proteins and Conformational Models for Prion Diseases....Pages 49-67 The Folding Intermediate Concept of Prion Protein Formation and Conformational Links to Infectivity....Pages 69-76 Cell Biology of the Prion Protein....Pages 77-93 Transgenetics and Gene Targeting in Studies of Prion Diseases....Pages 95-123 Transgenetics and Neuropathology of Prion Diseases....Pages 125-146 Evidence for Two Prions in Yeast: [URE3] and [PSI]....Pages 147-160 Back Matter....Pages 161-167

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