Bovine Spongiform Encephalopathy

1. Front Matter

   Pages i-xxv

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2. The Potential Risk to Humans of Amyloids in Animals

   1. The Potential Risk to Humans of Amyloids in Animals

      • D. Carleton Gajdusek

      Pages 1-7

3. Host Range and Pathogenesis

   1. Front Matter

      Pages 9-9

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   2. Bovine Spongiform Encephalopathy Distribution and Update on Some Transmission and Decontamination Studies

      • Ray Bradley

      Pages 11-27

   3. Preliminary Observations on the Pathogenesis of Experimental Bovine Spongiform Encephalopathy

      • Gerald A. H. Wells, Michael Dawson, Stephen A. C. Hawkins, Anthony R. Austin, Robert B. Green, Ian Dexter et al.

      Pages 28-44

   4. Recent Observations on the Epidemiology of Bovine Spongiform Encephalopathy

      • John W. Wilesmith

      Pages 45-55

4. Transmission and Pathogenesis of Spongiform Encephalopathies

   1. Front Matter

      Pages 57-57

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   2. Scrapie: Studies on Vertical and Horizontal Transmission

      • James L. Hourrigan, Albert L. Klingsporn

      Pages 59-83

   3. Transmission of Sheep and Goat Strains of Scrapie from Experimentally Infected Cattle to Hamsters and Mice

      • Clarence J. Gibbs Jr., Jiri Safar, Michael P. Sulima, Alfred E. Bacote, R. Andres San Martin

      Pages 84-91

   4. Experimental Transmission of Scrapie to Cattle

      • Randall C. Cutlip, Janice M. Miller, Richard E. Race, Allen L. Jenny, Howard D. Lehmkuhl, Mark M. Robinson

      Pages 92-96

   5. An Assessment of Transmissible Mink Encephalopathy as an Indicator of Bovine Scrapie in U.S. Cattle

      • Mark M. Robinson

      Pages 97-107

   6. Experimental Infections of Cattle and Mink with the Agents of Transmissible Mink Encephalopathy, Scrapie, and Bovine Spongiform Encephalopathy

      • Mark M. Robinson

      Pages 108-113

   7. BSE-Free Status: What Does It Mean?

      • Richard F. Marsh

      Pages 114-121

   8. Differing Neurohistologic Images of Scrapie, Transmissible Mink Encephalopathy, and Chronic Wasting Disease of Mule Deer and Elk

      • William J. Hadlow

      Pages 122-137

   9. Analysis of Risk Factors and Active Surveillance for BSE in Argentina

      • A. A. Schudel, B. J. Carrillo, E. L. Weber, J. Blanco Viera, E. J. Gimeno, C. Van Gelderen et al.

      Pages 138-154

   10. Speculations on the Origin of BSE and the Epidemiology of CJD

       • Richard H. Kimberlin

       Pages 155-175

5. Biochemistry: Protein Chemistry, Molecular Biology, and Molecular Genetics of the Spongiform Encephalopathies

   1. Front Matter

      Pages 177-177

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   2. Structure and Biologic Characteristics of Prion Protein (Scrapie Amyloid): Implications for the Safety of Naturally Derived Biologics

      • Jiri Safar

      Pages 179-191

   3. Prion Strains and Neuromuscular Disease in PrP Transgenic Mice

      • George A. Carlson

      Pages 192-201

   4. Deciphering Prion Diseases with Transgenic Mice

      • Glenn C. Telling, Michael Scott, Stanley B. Prusiner

      Pages 202-231

The very first international working discussion on slow infections of the nervous system was entitled "Slow, Latent, and Temperate Virus Infec­ tions" and was held at the National Institutes of Health (NIH) in December 1964. The primary impetus was the discovery and investigation of kuru in New Guinea by D. Carleton Gajdusek, M. D. This working discussion brought together investigators in human and veterinary medicine, virolo­ gists, microbiologists, and neuropathologists actively engaged in laboratory work with viruses that illustrated properties of latency, masking, slowness, or temperateness, with emphasis on subacute and chronic neurologic dis­ eases of unknown etiology. In the Preface to the monograph of published papers presented at the working discussion, Gajdusek and Gibbs wrote the following: After microbiology had given solution to the etiology of most acute infections of the central nervous system and after fungi and bacteria had been incriminated in impor­ tant chronic disorders of the nervous system such as torula and tuberculosis men­ ingitis, we have been left, in neurology, with a wide range of subacute and chronic affections of the central nervous systems of unknown etiology. Some of these diseases, still listed as idiopathic, are among the most prevalent afflictions of the central nervous system. Many others with familial patterns of occurrence do not yet have their basic pathogenesis or underlying metabolic defect elucidated, although we tend to think of them as genetically mediated.

• BSE
• biochemistry
• genetics
• infection
• infections
• molecular biology
• molecular genetics
• pathogenesis

• Laboratory of Central Nervous System Studies Basic Neurosciences Program Division of Intramural Research, National Institute of Neurological Disorders and Stroke National Institutes of Health, Bethesda, USA

  Clarence J. Gibbs

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