Novel Infectious Agents and the Central Nervous System

The term "unconventional virus" refers to virus-like agents that differ from conventional viruses in significant respects--resistance to agents that inactivate normal viruses, for example. Certain rare and fatal neurological disorders, such as the Creutzfeldt-Jacob syndrome and the Gerstmann-Straussler syndrome, are clearly produced by virus-like infectious agents, but the nature of these agents is not understood. This volume brings together the latest information on one such disease: Scrapie, a disease occurring in sheep. It discusses the nature of this infectious agent and insights gained through new techniques in molecular genetics. The work as a whole focuses on the implications of animal data for research on human disorders, such as the possible connection between unconventional viruses and Alzheimer's Disease. The discussions represent the work of specialists from a broad spectrum of fields including virology, neuropathology, and molecular biology.

Auteur
The Novartis Foundation is an international scientific and educational charity which promotes the study and general knowledge of science and in particular encourages international co-operation in scientific research.

Texte du rabat
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

Contenu
Partial table of contents:
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 al.).
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 Biology(S.
Prusiner, et al.).
Index of Contributors.
Subject Index.