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Wellcome at our non-profit website! We are dedicated to networking between prion experts, with the aim of effective containment of disease. Our principal activity is the publication of carefully edited interdisciplinary information on prion diseases. We also focuse on the history of research on prion diseases in humans and animals.

Preface by Werner Arber, Nobel Laureate

Table of Contents

Topic I: History
Topic II: Molecular Biology and Genetics
Topic III : Portraits of Prion Diseases
Topic IV: Pathology
Topic V: Surveillance, Clinical Aspects & Diagnostics
Topic VI: Epidemiology
Topic VII: Transmissibility
Topic VIII: Agent Inactivation
Topic IX: Prevention
Topic X: Risk Assessment

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of Prions in Humans and Animals


Prions in Humans and Animals


Topic I
History



1 Historical Introduction
B. Hörnlimann, D. Riesner, H. Kretzschmar, R. G. Will, S. C. MacDiarmid, G. A. H.
Wells, and M. P. Alpers

1.1 Introduction
1.2 The cause of prion diseases
1.3 Scrapie: archetype of all prion diseases
1.4 Transmissible mink encephalopathy
1.5 Chronic wasting disease in North American cervids
1.6 Creutzfeldt-Jakob disease and other human prion diseases
1.7 The scrapie-kuru connection
1.8 Etiological variety of prion diseases
1.9 New prion diseases
1.10 Prion diseases and contagion
1.11 Summary: traits common to all human and animal prion diseases
1.12 Synopsis of events, discoveries and findings since 1732


2 The History of Kuru Research
B. Hörnlimann
2.1 Introduction
2.2 From the stone age to the present: the Fore people
2.3 Discovery of kuru
2.4 The “Tukabu” ritual
2.5 The beginning of kuru research
2.6 A kuru hospital in Okapa
2.7 The spread of kuru
2.8 The pathological picture
2.9 The diet of the Fore
2.10 The geographical spread of the epidemic and the phylogenetic relations among kuru victims
2.11 The ancestral cult
2.12 Social impact
2.13 The discovery of transmissibility
2.14 The answers to the questions


3 History of Prion Research
S. B. Prusiner
3.1 Introduction
3.2 Animals and humans affected
3.3 In search of the cause
3.4 Amazing discovery
3.5 Prion diseases can be inherited
3.6 One protein, two shapes
3.7 Treatment ideas emerge
3.8 The mystery of “strains”
3.9 Breaking the barrier
3.10 The list may grow
3.11 Striking similarities


Topic II
Molecular Biology and Genetics

 
4 The Physical Nature of the Prion
D. Riesner
4.1 Introduction
4.2 The prion model and its nomenclature
4.3 The virus hypothesis
4.4 The virino hypothesis
4.5 The nucleic acid problem


5 Folding of the Recombinant Prion Protein
R. Glockshuber, J. Stöhr, and D. Riesner
5.1 Introduction
5.2 Folding of recombinant PrPc
5.3 The role of the single disulfide bond of PrP
5.4 Influence of point mutations linked with inherited human prion diseases on the thermodynamic stability of recombinant PrPc
5.5 Outlook


6 Structural Studies of Prion Proteins
S. Schwarzinger, D. Willbold, and J. Ziegler
6.1 Introduction
6.2 Structure of the globular domain of PrPc
6.3 Structural studies of full-length PrP
6.4 NMR studies on isolated structural features of PrP
6.5 High pressure NMR
6.6 Structural studies of PrPSc


7 Function of Cellular Prion Protein in Copper Homeostasis and Redox Signaling at the Synapse
J. Herms and H. Kretzschmar
7.1 Introduction
7.2 Cellular location of PrPc
7.3 Protein interactions with PrPc
7.4 PrPc binds copper(II) ions
7.5 Functional relevance of copper binding of PrPc at the synapse
7.6 Neuroprotective role of the prion protein in response to copper and oxidative stress
7.7 Redox signaling by PrPC modulates intracellular calcium homeostasis and synaptic function


8 The Scrapie Isoform of the Prion Protein PrPSc Compared to the Cellular Isoform PrPc
D. Riesner
8.1 Introduction
8.2 Biological and immunological properties of PrPSc
8.3 Chemical, biochemical, and physical properties of PrPSc
8.4 Structure of PrPSc
8.5 In vitro conversion of PrP and the generation of infectivity
8.6 Models of prion replication
8.7 Infectious, sporadic, and familial etiology of prion diseases


9 The Phylogeny of Mammalian and Nonmammalian Prion Proteins
H. M. Schätzl
9.1 Introduction
9.2 The organization of the PrP gene
9.3 Comparative analysis of PrP genes and prion proteins


10 Knockouts and Transgenic Mice in Prion Research
E. Flechsig, I. Hegyi, A. J. Raeber, A. Cozzio, A. Aguzzi, and C. Weissmann
10.1 Introduction
10.2 Generation and properties of PrP knockout mice
10.3 Transgenesis and gene replacement
10.4 Reverse genetics: studies on the structure–function relationship of PrP
10.5 Transgenic approaches to study intervention strategies against prion diseases
10.6 Investigating the mechanism of prion propagation by ectopic expression of PrP


11 Transplantation as a Tool in Prion Research
E. Flechsig, I. Hegyi, A. J. Raeber, A. Cozzio, A. Aguzzi, and C. Weissmann
11.1 Introduction
11.2 Prion-infected neurografts fail to cause neuropathological changes in PrP knockout mice
11.3 Spread of prions in the central nervous system requires PrPC-expressing tissue
11.4 Spread of prions from extracerebral sites to the CNS


12 Prion Strains
M. H. Groschup, A. Gretzschel, and T. Kuczius
12.1 Introduction
12.2 Definition of the term “prion strain”
12.3 Characteristics of prion strains
12.4 Adaptation and selection
12.5 Known prion strains in different species
12.6 Explanatory approaches with regard to the development and existence of prion strains
 

Topic III
Portraits of Prion Diseases


13 Portrait of Kuru
B. Hörnlimann and M. P. Alpers
13.1 History of kuru
13.2 Forms or variants of kuru
13.3 Incubation period, transmissibility, and susceptibility of kuru
13.4 Clinical signs of kuru and course of disease of kuru
13.5 Differential diagnoses of kuru
13.6 Epidemiology of kuru
13.7 Pathology of kuru
13.8 Is kuru a new disease?
13.9 Risk factors of kuru
13.10 Surveillance, prevention, and control of kuru
13.11 Editor’s note


14 Portrait of Creutzfeldt-Jakob Disease
H. Budka
14.1 History of CJD
14.2 Forms or variants of Creutzfeldt-Jakob Disease
14.3 Incubation period, transmissibility, and susceptibility of CJD 
14.4 Clinical signs of CJD and course of disease
14.5 Differential diagnoses of CJD 
14.6 Epidemiology of CJD
14.7 Pathology of CJD
14.8 Is CJD a new disease?
14.9 Risk factors of CJD 
14.10 Surveillance, prevention, and control of CJD


15 Portrait of Variant Creutzfeldt-Jakob Disease
R. G. Will and J. W. Ironside
15.1 History of vCJD
15.2 Forms or variants
15.3 Incubation period, transmissibility, and susceptibility of vCJD
15.4 Clinical signs of vCJD and course of disease
15.5 Differential diagnoses of vCJD
15.6 Epidemiology of vCJD
15.7 Pathology of vCJD
15.8 Is vCJD a new disease?
15.9 Risk factors of vCJD
15.10 Surveillance, prevention, and control of vCJD


16 Portrait of Gerstmann-Sträussler-Scheinker Disease
H. Budka
16.1 History of GSS
16.2 Forms or variants of GSS
16.3 Incubation period, transmissibility, and susceptibility of GSS
16.4 Clinical signs of GSS and course of disease
16.5 Differential diagnoses of GSS 
16.6 Epidemiology of GSS
16.7 Pathology of GSS
16.8 Is GSS a new disease?
16.9 Risk factors of GSS 
16.10 Surveillance, prevention, and control of GSS 


17 Portrait of Fatal Familial Insomnia and Sporadic Fatal Insomnia
H. Budka and E. Gelpi
17.1 History of FFI and SFI
17.2 Forms or variants of FFI and SFI
17.3 Incubation period, transmissibility, and susceptibility of FFI and SFI
17.4 Clinical signs of FFI and SFI and course of disease
17.5 Differential diagnoses of FFI and SFI
17.6 Epidemiology of FFI and SFI
17.7 Pathology of FFI and SFI
17.8 Are FFI and SFI new diseases?
17.9 Risk factors of FFI and SFI
17.10 Surveillance, prevention, and control of FFI and SFI


18 Portrait of Scrapie in Sheep and Goat
B. Hörnlimann, L. v. Keulen, M. J. Ulvund, and R. Bradley
18.1 History of scrapie
18.2 Forms or variants of scrapie agent strains
18.3 Incubation period, transmissibility, and susceptibility of scrapie
18.4 Clinical signs of scrapie and course of disease
18.5 Differential diagnoses of scrapie
18.6 Epidemiology of scrapie
18.7 Pathology of scrapie
18.8 Is scrapie a new disease?
18.9 Risk factors
18.10 Surveillance, prevention, and control of scrapie


19 Portrait of Bovine Spongiform Encephalopathy in Cattle and Other Ungulates
B. Hörnlimann, J. Bachmann, and R. Bradley
19.1 History of BSE
19.2 Forms or variants of BSE
19.3 Incubation period, transmissibility, and susceptibility of BSE
19.4 Clinical signs of BSE and course of disease
19.5 Differential diagnoses of BSE
19.6 Epidemiology of BSE
19.7 Pathology and Pathogenesis of BSE
19.8 Is BSE a new diseases?
19.9 Risk factors of BSE
19.10 Surveillance, prevention, and control of BSE


20 Portrait of Prion Diseases in Zoo Animals
J. K. Kirkwood and A. A. Cunningham
20.1 History 
20.2 Forms or variants of prion diseases in zoo animals
20.3 Incubation period, transmissibility, and susceptibility
20.4 Clinical signs and course of disease
20.5 Differential diagnoses
20.6 Epidemiology
20.7 Pathology
20.8 Are prion diseases of zoo animals new?
20.9 Risk factors
20.10 Surveillance, prevention, and control


21 Portrait of Chronic Wasting Disease in Deer Species
E. S. Williams† and M. W. Miller
21.1 History of CWD 
21.2 Forms or variants of CWD 
21.3 Incubation period, transmissibility, and susceptibility of CWD 
21.4 Clinical signs of CWD and course of disease
21.5 Differential diagnoses of CWD 
21.6 Epidemiology of CWD 
21.7 Pathology of CWD 
21.8 Is CWD a new prion disease?
21.9 Risk factors of CWD 
21.10 Surveillance, prevention, and control of CWD 


22 Portrait of Transmissible Mink Encephalopathy
W. J. Hadlow
22.1 History of TME
22.2 Forms or variants of TME
22.3 Incubation period, transmissibility, and susceptibility of TME
22.4 Clinical signs of TME and course of disease
22.5 Differential diagnoses of TME
22.6 Epidemiology of TME
22.7 Pathology of TME
22.8 Is TME a new disease?
22.9 Risk factors of TME
22.10 Surveillance, prevention, and control of TME


23 Portrait of Transmissible Feline Spongiform Encephalopathy
M. Hewicker-Trautwein and R. Bradley
23.1 History of FSE 
23.2 Forms or variants of FSE
23.3 Incubation period, transmissibility, and susceptibility of FSE
23.4 Clinical signs of FSE and course of disease
23.5 Differential diagnoses of FSE
23.6 Epidemiology of FSE
23.7 Pathology of FSE
23.8 Is FSE a new disease?
23.9 Risk factors of FSE
23.10 Surveillance, prevention, and control of FSE


24 Portrait of Experimental BSE in Pigs
G. A. H. Wells, S. A. C. Hawkins, J. Pohlenz†, and D. Matthews
24.1 History of BSE in pigs
24.2 Forms or variants of experimental BSE in pigs
24.3 Incubation period, transmission, and susceptibility
24.4 Clinical signs and course of disease
24.5 Differential diagnoses
24.6 Epidemiology
24.7 Pathology of BSE in pigs
24.8 Is there a TSE of pigs?
24.9 Risk factors 
24.10 Surveillance, prevention, and control


25 Portrait of a Spongiform Encephalopathy in Birds and the Transmissibility of Mammalian Prion Diseases to Birds
G. A. H. Wells, J. Pohlenz†, S. A. C. Hawkins, and D. Matthews
25.1 History of prion disease in ostriche (?)
25.2 Avian prion diseases?
25.3 Transmission studies on prion diseases in birds
25.4 Clinical signs and course of the SE of ostriches
25.5 Differential diagnoses of the SE of ostriches
25.6 Epidemiology of the SE of ostriches
25.7 Pathology of the SE of ostriches
25.8 What is the significance of the SE of ostriches?
25.9 Risk factors and prevention of prion disease in birds
25.10 Surveillance of TSE in birds 


Topic IV
Pathology



26 Pathology and Genetics of Human Prion Diseases
H. Kretzschmar and P. Parchi
26.1 Introduction
26.2 Neuropathologic features of prion diseases in humans
26.3 PrPSC in non-neuronal tissues
26.4 The human prion protein and prion protein gene (PRNP)
26.5 Neuropathological phenotypes of human prion diseases
26.6 Genetics of human prion diseases: phenotypes of familial (genetic) prion diseases


27 The Pathology of Prion Diseases in Animals
G. A. H. Wells, S. J. Ryder, and W. J. Hadlow
27.1 Introduction
27.2 Pathology of scrapie in sheep and goats
27.3 Pathology of transmissible mink encephalopathy (TME)
27.4 Pathology of chronic wasting disease
27.5 Pathology of bovine spongiform encephalopathy
27.6 Pathology of BSE in non domestic captive ungulate species
27.7 Pathology of feline spongiform encephalopathy


28 Pathophysiology of Prion Diseases Following Peripheral Infection
W. J. Schulz-Schaeffer, H. Kretzschmar, and M. Beekes
28.1 Introduction
28.2 Administration of the pathogen in animal experiments
28.3 Cell culture experiments
28.4 Peripheral paths of infection
28.5 Significance of the hematopoetic system, in particular the spleen
28.6 Species-specific differences
28.7 Neuronal spread of infection to the central nervous system


Topic V
Surveillance, Clinical Aspects and Diagnostics



29 Introduction to the Surveillance of Human Prion Diseases
B. Hörnlimann, H. Kretzschmar, R. G. Will, O. Windl, and H. Budka
29.1 Introduction
29.2 Surveillance of patients on an out-patient and in-patient basis
29.3 Surveillance in neuropathological laboratories and diagnostic laboratories
29.4 International cooperation and the importance of national health authorities
29.5 Epidemiological surveillance and case control studies
29.6 The problem of possible phenotypic variation of disease caused by BSE prions in humans


30 Clinical Findings in Human Prion Diseases
M. Sturzenegger and R. G. Will
30.1 Introduction
30.2 Differential diagnosis and additional paraclinical investigations
30.3 Clinical features of Creutzfeldt–Jakob disease
30.4 Clinical features of variant CJD
30.5 Clinical features of Gerstmann–Sträussler–Scheinker disease
30.6 Clinical features of fatal familial insomnia
30.7 Clinical features of sporadic fatal insomnia
30.8 Clinical features of kuru
30.9 Annex: historical classification


31 Clinical Diagnostic Methods for the Diagnosis of Human Prion Diseases
I. Zerr
31.1 Introduction
31.2 Electroencephalogram
31.3 Analysis of cerebrospinal fluid
31.4 Imaging techniques
31.5 Sensitivity of clinical diagnostic tests in distinct molecular CJD subtypes
31.6 Diagnostic procedure


32 Introduction to Surveillance of Animal Prion Diseases
B. Hörnlimann, M. G. Doherr, D. Matthews, and S. C. MacDiarmid
32.1 Introduction
32.2 Passive surveillance of animal prion diseases
32.3 “Active” surveillance of animal prion diseases


33 Clinical Findings in Bovine Spongiform Encephalopathy
E. Schicker, U. Braun, B. Hörnlimann, and T. Konold
33.1 Introduction
33.2 Clinical history and course of the disease
33.3 Differential diagnosis of BSE
33.4 General clinical examination findings
33.5 Neurological examination findings
33.6 Laboratory findings
33.7 Examination at abattoirs – a diagnostic challenge


34 Clinical Findings in Scrapie
M. J. Ulvund
34.1 Introduction
34.2 Case history
34.3 Findings of the general clinical examination
34.4 Differential diagnoses
34.5 Course of the disease
34.6 Findings of neurological examinations
34.7 Laboratory findings


35 Diagnosis of Bovine Spongiform Encephalopathy by Immunological Methods
A. J. Raeber, M. Moser, and B. Oesch
35.1 Introduction
35.2 Properties of the normal and disease-associated form of the prion protein
35.3 Rapid tests approved by European authorities
35.4 Ante mortem TSE test development
35.5 Identification of atypical BSE strains


Topic VI
Epidemiology



36 Epidemiology and Risk Factors of Creutzfeldt-Jakob Disease
I. Zerr and S. Poser†
36.1 Introduction
36.2 Descriptive epidemiology
36.3 Risk factors for sporadic CJD
36.4 Epidemiology and risk factors of acquired forms of CJD


37 Creutzfeldt-Jakob Disease in Germany
I. Zerr, S. Poser†, and H. Kretzschmar
37.1 Introduction
37.2 German CJD surveillance study
37.3 CJD epidemiology in Germany
37.4 Prognostic factors in sporadic CJD


38 The Epidemiology of Kuru
M. P. Alpers and B. Hörnlimann
38.1 Introduction
38.2 Frequency of cases and progression of the epidemic 
38.3 The geographical spread and related cultural events 
38.4 Distribution according to sex and age 
38.5 Socio-cultural background of the sex- and age-specific distribution 
38.6 Explanation for the survival time curve (time of infection and infectious dose) 
38.7 Explanation of kuru cases among children (modes of infection) 
38.8 Conclusion and present significance of kuru 


39 The Course of the BSE Epidemic – Retrospective Epidemiological Considerations
B. Hörnlimann, Judith B. Ryan, and S. C. MacDiarmid
39.1 Introduction 
39.2 Basic epidemiological data on BSE in the UK 
39.3 The factors that determined the course of the epidemic 


40 The Causes of the BSE Epidemic
S. Dahms and B. Hörnlimann
40.1 Introduction
40.2 The case-series study: development of the feed borne hypothesis
40.3 The case-control study: investigations on the feed borne hypothesis


Topic VII
Transmissibility


41 The Experimental Transmissibility of Prions and Infectivity Distribution in the Body
M. H. Groschup, M. Geissen and A. Buschmann
41.1 Introduction
41.2 Brief historical overview
41.3 Design of experimental transmission studies
41.4 Experimental transmissibility of human prion diseases
41.5 Experimental transmissibility of animal prion diseases
41.6 Infectivity distribution in peripheral organs
41.7 Pathegonesis studies


42 The Iatrogenic and “Natural” Transmissibility of Prion Diseases
M. H. Groschup, B. Hörnlimann, and A. Buschmann
42.1 Introduction
42.2 Natural transmission within one species
42.3 Natural transmission of scrapie to other species
42.4 Natural transmission of BSE to other species
42.5 Iatrogenic transmission in human and veterinary medicine
42.6 Genetically determined susceptibility 


Topic VIII
Agent Inactivation


43 Inactivation in Practice – Risk Assessment and Validation for Food Gelatin
S. C. MacDiarmid
43.1 Introduction
43.2 Raw materials
43.3 Dilution
43.4 Acid treatment
43.5 Alkaline treatment
43.6 Further acid treatment
43.7 Extraction of gelatine
43.8 Experimental studies
43.9 Conclusions


44 Chemical Disinfection and Inactivation of Prions
B. Hörnlimann, W. J. Schulz-Schaeffer, K. Roth, Zheng-Xin Y., H. Müller, R. C. Oberthür and D. Riesner
44.1 Introduction
44.2 Basic knowledge regarding the chemical inactivation of prions
44.3 Prerequisites for the efficiency of chemical disinfactants
44.4 Testing for prion depletion and inactivation efficiency
44.5 Chemical disinfactants suitable for the inactivation of prions
44.6 Chemical disinfactants unsuitable or less suitable for the inactivation of prions


45 Thermal Inactivation of Prions
R. C. Oberthür, H. Müller, and D. Riesner
45.1 Introduction
45.2 Physical chemistry of heat inactivitation of complex biological structures
45.3 Kinetics of thermal denaturation and inactivation
45.4 Experimental setup for the thermal inactivation of a specimen
45.5 Results of inactivation studies
45.6 Inactivation of prions under oleochemical conditions
45.7 Practical and theoretical implications


Topic IX
Prevention



46 Prevention of Prion Diseases in the Production of Medicinal Products, Medical Devices, and Cosmetics
M. Ruffing, H. Windemann, and J. Schäfer
46.1 Introduction
46.2 Regulations to prevent the transmission of prion diseases by medicinal products and cosmetics
46.3 Evaluation of the risk of medicinal products transmitting prion diseases
46.4 Regulations for specific materials used in the production of medicinal products
46.5 Regulations to prevent the transmission of prion diseases by medical devices


47 Prevention of the Transmission of Prion Diseases in Healthcare Settings
B. Hörnlimann, G. Pauli, K. Lemmer, M. Beekes, and M. Mielke
47.1 Introduction
47.2 Patient care
47.3 Risk of accidental occupational transmission in nosocomial and other healthcare settings
47.4 Iatrogenic transmission of humans TSEs: retrospective findings and current risk assessment
47.5 Preventive measures for handling CSF and tissue samples
47.6 Transmission through blood and blood products
47.7 Precautionary measures to minimize the risk of transmission via surgical interventions on patients with an evident or potential risk of CJD or vCJD
47.8 Disinfection and sterilization of instruments and materials
47.9 Decontamination of instruments following surgery on patients without any specific signs or symptoms pointing to a risk of transmission
47.10 Prevention in specific areas
47.11 Handling of corpses prior to interment
47.12 Waste disposal in hospitals and laboratories


48 Precautionary Measures for Autopsies Performed in Cases of  Suspected Prion Disease
W. J. Schulz-Schaeffer, A. Giese, and H. Kretzschmar
48.1 Introduction
48.2 Performing the autopsy
48.3 Decontamination and resistance of the infectious agent


49 Prevention of Prion Diseases in Research Laboratories
A. J. Raeber and A. Aguzzi
49.1 Introduction
49.2 Risk categorization of prions
49.3 Risk assessment for work with prions and prion proteins
49.4 Risk classification for work with prions and prion proteins
49.5 Containment of laboratory work with prions
49.6 Inactivation of prions in research laboratories
49.7 Post-exposure prophylaxis following spills and accidents
49.8 Further useful information 
 

Topic X
Risk Assessment



50 Evidence for a Link between the New Variant of Creutzfeldt-Jakob Disease and Bovine Spongiform Encephalopathy
M. E. Bruce, R. G. Will, J. W. Ironside, and H. Fraser
50.1 Introduction
50.2 TSE strain discrimination in mice
50.3 Transmissions of Animal TSEs to mice
50.4 Transmissions of vCJD and sCJD to mice
50.5 Conclusions


51 Risk Assessment of Transmitting Prion Diseases through Blood, Cornea, and Dura Mater
J. Löwer and Th. R. Kreil
51.1 Introduction
51.2 Blood
51.3 Cornea
51.4 Dura Mater


52 BSE Risk Assessment and Minimization
R. C. Oberthür, A. A. de Koeijer, B. E. C. Schreuder, and S. C. MacDiarmid
52.1 Introduction
52.2 Definition of the term “risk”
52.3 Dose-response relationship in BSE
52.4 Reproduction number
52.5 Reproduction number in Great Britain over time
52.6 BSE risk minimization within the cattle population
52.7 BSE risk minimization from cattle to humans
52.8 Control of the efficiency of BSE risk minimization


53 BSE control – Internationally Recommended Approaches
S. C. MacDiarmid, P. Infanger, and B. Hörnlimann
53.1 Introduction
53.2 Measures in response to the first case of BSE in a country
53.3 Measures for surveillance, prevention, and control of a BSE epidemic


54 Atypical Scrapie-Nor98
S. L. Benestad and B. Bratberg
54.1 Introduction
54.2 TSE surveillance program launched for small ruminants
54.3 Particularity of clinical signs of atypical scrapie
53.4 Particularity of genetics
54.5 Particularity of the pathology
54.6 Particularity of the diagnosis
54.7 The origin of atypical scrapie


55 Scrapie Control – Internationally Recommended Approaches
M. G. Doherr and N. Hunter
55.1 Introduction
55.2 Criteria to asses the scrapie status of a country or region
55.3 Disease monitoring
55.4 Measures to control scrapie in a country or region
55.5 Historical scrapie situation and potential import routes
55.6 National animal identification and tracing system
55.7 Genetic influences


56 The PrP Genotype as a Marker for Scrapie Susceptibility in Sheep
N. Hunter and A. Bossers 
56.1 Introduction
56.2 Sheep PrP gene (Prnp) and its variantions
56.3 Sheep PrP genotypes and association with susceptibility to TSEs
56.4 Methods of genotyping sheep


57 Scrapie Control at the National Level: The Norwegian Example
K. R. Alvseike, I. Melkild, and K. Thorud
57.1 Introduction
57.2 Number of scrapie cases in Norway
57.3 Scrapie surveillance
57.4 Control and eradication of scrapie
57.5 Scrapie prevention


Epilogue (by Colin L. Masters)

Appendix 1: Major Categories of Infectivity (WHO) - Tables IA, IB, IC

Appendix 2: Bovine Spongiform Encephalopathy (OIE): Animal Health Code - Chapter 2.3.13

Appendix 3: Scrapie (OIE): Animal Health Code - Chapter 2.4.8

Authors Index

Subject Index


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