Contents: Clinical Neurology and Epidemiology of Creutzfeldt-Jakob Disease, with Special Reference to Iatrogenic Cases. Neuropathology of Unconventional Virus Infections: Molecular Pathology of Spongiform Change and Amyloid Plaque Deposition, and more.
Novel Infectious Agents and the Central Nervous System Chairman: F. Brown 1988 Certain neurological disorders, such as Creutzfeldt-Jakob disease, are clearly caused by an infectious agent, but the nature of this agent has yet to be determined. Scrapie is a disease of sheep that shows the same properties as these rare but fatal human diseases. A cardinal feature of the disorders is the exceptionally long incubation period, which makes investigation of the causative agent a long-term and costly process. Studies have been greatly facilitated by the experimental transmission of scrapie to rodents, and the isolation of strains of scrapie that have relatively short incubations. The elusive nature of the scrapie agent (it is extremely resistant to normal physicochemical methods of inactivation) has meant that numerous hypotheses have been proposed for its structure. The basic issue is whether it contains a genome encoding sufficient information to direct its own replication, and the nature of this putative informational molecule. This book presents the latest results on scrapie, including classical genetic studies, characterization of the major protein found in scrapie-associated fibrils, and experiments using the cloning and gene transfer techniques of molecular biology. The implications of the findings from animal studies for understanding the human disorders are explored, and the neuropathology in both animals and humans is described. There is also a report on the epidemiology of the human diseases, and a discussion of the various features shared by these infectious neurological disorders and Alzheimer's disease. Recent Ciba Foundation books: No 126 Selective neuronal death Chairman: H. Wisniewski ISBN 0 471 91092 9 No 128 Novel diarrhoea viruses Chairman: R. F. Bishop ISBN 0 471 91094 5 No 129 Autoimmunity and autoimmune disease Chairman: D. K. Peters ISBN 0 471 91095 3 No 130 Molecular approaches to human polygenic disease Chairman: D. J. Weatherall ISBN 0 471 91096 1
Table of Contents
PARTIAL TABLE OF CONTENTS: Partial table of
Clinical Neurology and Epidemiology of
Creutzfeldt-Jakob Disease, with Special
Reference to Iatrogenic Cases (P. Brown).
Pathogenesis of Experimental Scrapie 37 (R.
Kimberlin C. Walker).
Genetic Control of Prion Incubation Period in
Mice (C. Carlson, et al.).
Potential Involvement of Retroviral Elements
in Human Dementias (L. Manuelidis, et al.).
Scrapie-Associated Fibrils, PrP Protein and
the Sinc Gene (J. Hope N. Hunter).
Properties of Scrapie Prion Proteins in
Liposomes and Amyloid Rods (R. Gabizon, et
Search for a Scrapie-Specific Nucleic Acid: A
Progress Report (B. Oesch, et al.).
Novel Mechanisms of Degeneration of the
Central Nervous System--Prion Structure and
Prusiner, et al.).
Index of Contributors.