Prion Diseases, Softcover reprint of the original 1st ed. 1996 Methods in Molecular Medicine Series, Vol. 3

Harry Baker and Rosalind Ridley have done an admirable job in assem­ bling this collection of articles that describe the methodology frequently used to study a group of CNS illnesses often referred to as the "prion diseases." Research on prions and the disorders that they cause has progressed relatively rapidly over the last decade since the discovery of the prion protein (PrP) that allowed the application of modem molecular biological and genetic tools. The power of these techniques is awesome and their use in deciphering the once mysterious prion diseases has brought a wealth of new information. Although prions are unprecedented pathogens, appearing to consist only of PrPSc molecules, the diseases that they cause are no less remarkable. The prion diseases in animals include scrapie of sheep and goats as well as "mad cow" disease or bovine spongiform encephalopathy (BSE). In the United King­ dom, the epidemic of BSE has heightened public awareness of this previously obscure group of diseases such that any work in the field is likely to stir up interest in the media and become a subject of public debate. It has been diffi­ cult for British investigators to work on prion diseases without being involved in these controversies. As such, several chapters have been included that deal with political and social issues surrounding prion diseases. The human prion diseases present an equally fascinating saga in which these CNS degenerations present as genetic, sporadic, and infectious illnesses.

The Paradox of Prion Disease. Human Spongiform Encephalopathy: Clinical Presentation and Diagnostic Tests. Neuropathological Diagnosis of Human Prion Disease: Morphological Studies. Neuropathological Diagnosis of Human Prion Disease: PrP Immunocytochemical Techniques. The Diagnosis of Bovine Spongiform Encephalopathy (BSE) and Scrapie by Detection of BSE Fibrils or Scrapie-Associated Fibrils (SAF) by Transmission Electron Microscopy and of the Abnormal Protein Isoform (PrPSc) by Immunoblotting. Exposure to, and Inactivation of, the Unconventional Agents that Cause Transmissible Degenerative Encephalopathies. Surveillance of Prion Diseases in Humans. Environmental Causes of Human Spongiform Encephalopathy. Bovine Spongiform Encephalopathy: Methods of Analyzing the Epidemic in the United Kingdom. Handling the BSE Epidemic in Great Britain. Special Problems of Genetic Counseling in Adult Onset Diseases: Huntington's Disease as a Model. Genotyping and Susceptibility of Sheep to Scrapie. Strain Typing Studies of Scrapie and BSE. PrP-Deficient Mice in the Study of Transmissible Spongiform Encephalopathies. Transgenic Approaches to Prion 'Species-Barrier' Effects. Methods for Studying Prion Protein (PrP) Metabolism and the Formation of Protease-Resistant PrP in Cell Culture and Cell-Free Systems. Immunohistochemistry of Resinated Tissues for Light and Electron Microscopy. Index.