Prions

 Table of Content

I. SCOPE AND METHODOLOGY	1
2. REPORT SYNOPSIS	3
2.1 Prions	3
Introduction	3
Exhibit 1. Mammalian Agents of Spongiform Encephalopathies, ICTVdb Code, Disease Name,  Natural Host, Prion Name and PrP Iso Form	4
Special Characteristics of Prions	4
Structure and Organization of Prions	5
Hypothesis	5
Prion Replication	5
Prion Diseases	6
Transmissible Spongiform Encephalopathy	6
Characteristics of Transmissible Spongiform Encephalopathy (TSE)	8
Bovine Spongiform Encephalopathy (BSE)	8
Exhibit 2. History of BSE	8
CJD	10
Major Types of CJD	10
Sporadic CJD	10
Hereditary CJD	11
Iatrogenic CJD	11
Variant CJD	11
Relationship between CJD and BSE	11
Scrapie	11
Kuru	12
Transmissible Mink Encephalopathy (TME)	12
Feline Spongiform Encephalopathy (FSE)	12
Chronic Wasting Disease (CWD)	12
Gerstmann-Sträussler-Scheinker Syndrome (GSS)	12
Control of prion Disease	13
Animal Feed	13
Animal Feed Regulations	14
Concern for Quality of Feed Ingredients with Increase in BSE around the World	14
Eurofins Forms Strategic Alliance with VLA in UK to Maintain Feed Safety	14
Plan on Ban of certain Cattle Material from Medical Products to Protect Against BSE	14
Role of WHO in TSE Control	15
Role of OIE	15
Prion Inactivation	16
Prion Disease Testing	17
2.2 Prions - Global Market Analysis	20
Exhibit 3. Prion Deactivation Technologies - Global Market Estimations & Predictions (2005 - 2020) in US$ Million	20
Exhibit 4. Prion Reduction Filters - Global Market Estimations & Predictions (2005 - 2020) in US$ Million	21
Exhibit 5. BSE Post Mortem Testing - Global Market Estimations & Predictions (2005 - 2020) in US$ Million	22
Exhibit 6. List of Global Major Prions Companies	23
3. MARKET DYNAMICS	24
3.1 World Cattle Demographics	24
Exhibit 7. World Cattle Population (2000-2012) – By Geographic Regions for North America, Europe, Japan and Rest of World in Million Head	24
3.2 Market Overview	25
3.3 Global Prion Deactivation Technologies Market	26
Prion Deactivation Technologies - Global Market Analysis	26
Exhibit 8. Prion Deactivation Technologies - Global Market Estimations & Predictions (2005 - 2020) by Geographic Regions for North America, Europe, Japan and Rest of World in US$ Million	26
Exhibit 9. Prion Deactivation Technologies - Global Market Shares (2010, 2015 & 2020) by Geographic Regions for North America, Europe, Japan and Rest of World	27
3.4 North American Prion Deactivation Technologies Market	28
Exhibit 10. Prion Deactivation Technologies - North American Market Estimations & Predictions (2005 - 2020) by Geographic Region for Canada and United States in US$ Million	28
Exhibit 11. Prion Deactivation Technologies - North American Market Shares (2010, 2015 & 2020) by Geographic Region for Canada and United States	29
3.4 (I) United States Prion Deactivation Technologies Market	29
3.4 (II) Canadian Prion Deactivation Technologies Market	29
Exhibit 12. List of Major Companies in North America	29
3.5 European Prion Deactivation Technologies Market	31
Exhibit 13. Prion Deactivation Technologies - European Market Estimations & Predictions (2005 - 2020) by Geographic Region for United Kingdom, France, Ireland, Portugal, Switzerland, Germany, Russia and Rest of Europe in US$ Million	31
Exhibit 14. Prion Deactivation Technologies - European Market Shares (2010, 2015 & 2020) by Geographic Region for United Kingdom, France, Ireland, Portugal, Switzerland, Germany, Russia and Rest of Europe	32
3.5 (I) United Kingdom Prion Deactivation Technologies Market	32
3.5 (II) French Prion Deactivation Technologies Market	32
3.5 (III) Irish Prion Deactivation Technologies Market	32
3.5 (IV) Portuguese Prion Deactivation Technologies Market	32
3.5 (V) Swiss Prion Deactivation Technologies Market	33
3.5 (VI) German Prion Deactivation Technologies Market	33
3.5 (V) Russian Market for Prion Deactivation Technologies	33
3.6 Japanese Prion Deactivation Technologies Market	34
Exhibit 15. Prion Deactivation Technologies - Japanese Market Estimations & Predictions (2005 - 2020) in US$ Million	34
3.7 Rest of World Prion Deactivation Technologies Market	35
Exhibit 16. Prion Deactivation Technologies – Rest of World Market Estimations & Predictions (2005 - 2020) by Geographic Region for Israel, South Africa, Brazil and Other Countries in US$ Million	35
Exhibit 17. Prion Deactivation Technologies - Rest of World Market Shares (2010, 2015 & 2020) by Geographic Region for Israel, South Africa, Brazil and Other Countries	36
3.6 (I) Israeli Prion Deactivation Technology Market	36
3.6 (II) South African Prion Deactivation Technology Market	36
3.6 (III) Brazilian Prion Deactivation Technology Market	36
3.10 Global Prion Reduction Filters Market	37
Prion Reduction Filters – Global Market Analysis	37
Exhibit 18. Prion Reduction Filters - Global Market Estimations & Predictions (2005 - 2020) by Geographic Regions for Europe, North America, Japan and Rest of World in US$ Million	38
Exhibit 19. Prion Reduction Filters - Global Market Shares (2010, 2015 & 2020) by Geographic Region for Europe, North America, Japan and Rest of World	39
3.11 North American Prion Reduction Filters Market	40
Exhibit 20. Prion Reduction Filters - North American Market Estimations & Predictions (2005 - 2020) by Geographic Region for Canada and United States in US$ Million	40
Exhibit 21. Prion Reduction Filters - North American Market Shares (2010, 2015 & 2020) by Geographic Region for Canada and United States	41
3.11 (I) United States Prion Reduction Filters Market	41
3.11 (II) Canadian Prion Reduction Filters Market	41
3.12 European Prion Reduction Filters Market	42
Exhibit 22. Prion Reduction Filters - European Market Estimations & Predictions (2005 - 2020) by Geographic Regions for United Kingdom, France, Ireland, Portugal, Switzerland, Germany, Russia and Rest of Europe in US$ Million	42
Exhibit 23. Prion Reduction Filters - European Market Shares (2010, 2015 & 2020) by Geographic Region for United Kingdom, France, Ireland, Portugal, Switzerland, Germany, Russia and Rest of Europe	43
3.12 (I) United Kingdom Prion Reduction Filters Market	43
3.12 (II) French Prion Reduction Filters Market	43
3.12 (III) Irish Prion Reduction Filters Market	43
3.12 (IV) Portuguese Prion Reduction Filters Market	43
3.12 (V) Swiss Prion Reduction Filters Market	43
3.12 (VI) German Prion Reduction Filters Market	44
3.12 (VI) Russian Prion Reduction Filters Market	44
3.13 Japanese Prion Reduction Filters Market	45
Exhibit 24. Prion Reduction Filters - Japanese Market Estimations & Predictions (2005 - 2020) in US$ Million	45
3.14 Rest of World Prion Reduction Filters Market	46
Exhibit 25. Prion Reduction Filters - Rest of World Market Estimations & Predictions (2005 - 2020) by Geographic Region for Israel, South Africa, Brazil and Other Countries in US$ Million	46
Exhibit 26. Prion Reduction Filters – Rest of World Market Shares (2010, 2015 & 2020) by Geographic Region for by Geographic Region for Israel, South Africa, Brazil and Other Countries	47
3.11.1.7 (II) Israel Prion Reduction Filters Market	47
3.11.1.7 (II) South Africa Prion Reduction Filters Market	47
3.11.1.7 (II) Brazil Prion Reduction Filters Market	47
3.15 Global BSE Post Mortem Testing Market	48
BSE Post Mortem Testing – Global Market Analysis	48
Exhibit 27. BSE Post Mortem Testing - Global Market Estimations & Predictions (2005 - 2020) by Geographic Regions for Europe, North America, Japan and Rest of World in US$ Million	49
Exhibit 28. BSE Post Mortem Testing - Global Market Shares (2010, 2015 & 2020) by Geographic Region for Europe, North America, Japan and Rest of World	50
Exhibit 29. List of Countries with BSE Risk Status	50
3.16 North American BSE Post Mortem Testing Market	52
Exhibit 30. BSE Post Mortem Testing - North American Market Estimations & Predictions (2005 - 2020) by Geographic Region for Canada and United States in US$ Million	52
Exhibit 31. BSE Post Mortem Testing - North American Market Shares (2010, 2015 & 2020) by Geographic Region for Canada and United States	53
Exhibit 32. Number of Confirmed BSE cases in North America (2000 and before – 2013)	53
3.16 (I) United States BSE Post Mortem Testing Market	54
US Allocation of Funds for Food Safety and BSE Research	54
Harmonization of BSE Management Policies by United States, Canada, and Mexico	54
Upgrading of BSE Risk Status	55
3.16 (II) Canadian BSE Post Mortem Testing Market	55
3.17 European BSE Post Mortem Testing Market	56
Exhibit 33. BSE Post Mortem Testing - European Market Estimations & Predictions (2005 - 2020) by Geographic Regions for United Kingdom, France, Ireland, Portugal, Switzerland, Germany, Russia and Rest of Europe in US$ Million	56
Exhibit 34. BSE Post Mortem Testing - European Market Shares (2010, 2015 & 2020) by Geographic Region for United Kingdom, France, Ireland, Portugal, Switzerland, Germany, Russia and Rest of Europe	57
Exhibit 35. Number of Confirmed BSE cases in European Countries (2000 and before – 2013)	58
3.17 (I) European Cattle Demographics	59
Exhibit 36. Cattle – European Population (2000-2010) by Countries for UK, France, Ireland, Portugal, Switzerland, Germany and Rest of Europe in Million Head	59
European Policies on BSE	60
EU Commission’s Latest Proposal Reduces BSE Testing Costs	61
3.17 (II) United Kingdom BSE Post Mortem Testing Market	61
BSE Cases in the United Kingdom	61
Measures Taken by United Kingdom to Control BSE	62
3.17 (III) French BSE Post Mortem Testing Market	62
3.17 (IV) Irish BSE Post Mortem Testing Market	63
3.17 (V) Portuguese BSE Post Mortem Testing Market	63
3.17 (VI) Swiss BSE Post Mortem Testing Market	63
Swiss market for BSE Post Mortem Testing was estimated at US$24.59 million in 2012. The market is projected to reach US$108.79 million by 2020, growing at a CAGR of 21.29% during 2005-2020.	63
Swiss Beef Industry Awarded for Managing BSE	63
3.17 (VII) German BSE Post Mortem Testing Market	63
Germany to Modify BSE Testing Regulations	64
The Netherlands	64
New Case of Mad Cow Disease Reported in the Netherlands	64
Slovenia	64
Slovenian Authorities Confirm BSE Case Findings	64
Austria	65
August 2005, Third Case of Mad Cow Reported in Austria	65
Exhibit 37. List of Major Companies in Europe	65
3.18 Japanese BSE Post Mortem Testing Market	66
Exhibit 38. BSE Post Mortem Testing - Japanese Market Estimations & Predictions (2005 - 2020) in US$ Million	66
Exhibit 39. Number of Confirmed BSE Cases in Japan (2000 and before – 2013)	67
37th BSE Case Reported in Japan	67
BSE Cases Reported in Japan	67
Health Ministry Bans Blood Donations in Japan	68
A New Amendment Proposed by USDA on Import of Boneless Beef from Japan	68
Japan Re-enforces US Beef Ban	70
3.19 Rest of World BSE Post Mortem Testing Market	71
Exhibit 40. BSE Post Mortem Testing - Rest of World Market Estimations & Predictions (2005 - 2020) Geographic Region for Israel, South Africa, Brazil and Other Countries in US$ Million	71
Exhibit 41. BSE Post Mortem Testing - Rest of World Market Shares (2010, 2015 & 2020) by Geographic Region for Israel, South Africa, Brazil and Other Countries in US$ Million	72
Exhibit 42. Number of Confirmed BSE Cases in Rest of World (2000 and before – 2013)	73
3.11.1.2 (I) Israeli BSE Post Mortem Testing Market	73
3.11.1.2 (II) South African BSE Post Mortem Testing Market	73
3.11.1.2 (II) Brazilian BSE Post Mortem Testing Market	73
3.20 Major Companies in Prions Market	74
Analytik Jena AG (Germany)	74
Beckman Coulter, Inc. (USA)	76
Bio-Rad Laboratories, Inc. (USA)	76
Cayman Chemical Company (USA)	78
Covance, Inc. (USA)	78
Enfer Scientific (Ireland)	79
Eurofins Scientific SA (France)	81
Eurogentec SA (Belgium)	81
Gene Thera, Inc (USA)	82
GenWay Biotech, Inc. (USA)	82
IdentiGEN Ltd. (Ireland)	83
IDEXX Laboratories, Inc. (USA)	84
Meridian Life Science, Inc. (USA)	84
Microsens Biotechnologies (UK)	85
EMD Millipore (USA)	87
Neogen Corporation	88
Pall Corporation (UK)	89
Prionics AG (Switzerland)	89
ProMetic Life Sciences, Inc. (Canada)	90
Proteome Sciences plc (UK)	91
R-Biopharm AG (Germany)	92
ScheBo® • Biotech AG (Germany)	92
VMRD, Inc. (Veterinary Medical Research & Development) (USA)	92
3.21 Major Activities in Prions Industry	94
Bayer HealthCare Expands Animal Health Research Capacity	94
IDEXX Laboratories Acquires Madasa do Brasil Ltda.	94
Bayer HealthCare Acquires Teva’s U.S Animal Health Business	94
Macopharma Successfully Completes P-Capt Prion Studies	94
ProMetic Life Sciences in Association with Nantpharma Develops and Commercializes Plasma-Derived Biopharmaceuticals	95
Amorfix Collaborates with Global Leader in Prion Diagnostic Research	95
US$13.5 million Neurodegenerative Biomarker Consortium to be Lead by Karolinska	96
Scripps Scientists Discover Single Prion Protein Responsible for Neuronal Death	96
University of Alberta Researchers Discover Additional Link between Alzheimer's and Prion Diseases	97
IDEXX Laboratories Completes the Acquisition of RADIL Reference Laboratory from UoM (University of Missouri)	97
ProMetic Secures Binding Forecast from Octapharma for over US$ 2 million of Prion Capture Resin	98
PREVENT employs Amorfix Life Sciences’ ProMIS Technology	98
LGC Takes Over KBioscience	99
Drug Targets Discovered for Prion Diseases	99
High Affinity Antibodies to Cancer Specific Epitopes Successfully Produced by Amorfix	99
Prometric & MacoPharma Urges the DoH of UK to adopt P-Capt® filter for Safer (vCJD prion free) Blood Transfusion	100
Neogen Acquires GeneSeek	100
Chronix Biomedical Enters into Agreement with University of Calgary for Commercialization of Serum DNA based Blood Test BSE detection	101
ASP’s Sterrad Technology Receives AFSSAPS Approval for Complete Inactivation of Prions	101
New Blood Filter by Pall Corporation	102
ProMetic and Abraxis in Prion Capture Technology Commercialization Agreement	102
SaBTO Advises P-Capt® Prion Reduction Filter as Protection against vCJD Blood Transmission in Children	103
ProMetic Acquires ARC’s Stake in Pathogen Removal and Diagnostic Technologies Inc.	103
Evolutionary Origin of Prion Disease Gene Discovered	103
Beckman Coulter Acquires Cogenics	104
Bio-Rad TeSeE® SAP Test Gets USDA Approval	104
Alberta Building up Considerable Funds for Prion Research	105
Accelerated Method for the Detection of Infectious Prion Strains Developed by Scripps Florida	105
Sophisticated Techniques for Testing of Transmissible Spongiform Encephalopathies	106
BSE Testing Laboratory in Exeter by LGC	106
HemoBioTech’s Orth Technology Eradicates 100% Prions	107
BTG plc Acquires Protherics PLC	107
HPA Awarded Patent for Detection and Inactivation of Prions that Cause Variant Creutzfeldt - Jakob disease (vCJD)	108
INRA Establishes Animal House for Prion Research	108
Mysteries of Neurodegenerative Diseases Uncovered by the Royal Veterinary College.	109
British Blinded Panel of Spiked Human Blood Samples Results Released by Amorfix.	109
Resin for Prion Elimination in Plasma product for Biopharmaceutical Application Upgraded by ProMetic	110
Human Clinical Trials for P-Capt® Prion Filter Successfully Completed	110
The Concentration Technology of PRDT Magnifies Sensitivity of Diagnostic Tests for BSE	110
ProMetic BioTherapeutics Signs Agreement with Kedrion to Develop Multiple Hyperimmunes	111
Prionics Lelystad Launched in Netherlands	111
Eurofins Acquires Analycen	111
GeneThera and IZSLER in Collaboration Agreement	112
ProMetic Announces New Filter Product to Detect BSE in Cattle	112
Expanded Marketing of Mad Cow Disease Testing Kits by GeneThera	112
Private Testing for Mad Cow Disease Permitted by Court	113
Vita-Tech Acquired by IDEXX	113
IDEXX Acquires Critical Care Division of Osmetech	113
Covance now Becomes the Only Provider of Neuroscience Gold Standard Antibodies to Treat Alzheimer’s and Prions Diseases	113
Rapid BSE Diagnosis Facility of IdentiGEN Awarded ISO Certification	114
Abbott to Provide Blood Screening Facility Along with Four Important Tests to American Red Cross	114
Newly Developed Prion Capture Filter Receives CE Mark	115
IDEXX Acquires Golden Veterinary Laboratory in South Africa	115
New Acquisition Results in Comprehensive Antibody Products and Services	115
Prionics Buys CEDI Diagnostics, Creates New Strategic Alliance with World Class Science Innovation Center	115
Prionics Buys Pfizer Animal Health’s Diagnostic Products	116
IDEXX Acquires ITS Innovative Technologies and Systems	116
Covance Acquires GFI Research Center; Phase I Clinical Capacity Now Increased	117
Pall Corporation in Collaboration with Fresenius Kabi for Speedy Implementation of Prion Reduction Technology	117
CFIA Endorses New BSE Test	118
Strategic Alliance between ProMetic and Top Meadow to Develop New Diagnostics for the Easy Detection of BSE	118
Pall Launches Filter System to Eliminate Prions from Blood	118
Pall Corporation in Global Supply Agreement with Gambro BCT for Leukoreduction Filters	119
HerdChek® BSE Antigen EIA Test Kit from IDEXX Approved by European Commission	119
Euroflow Acquired by Pall Corporation	119
3.22 Beef Market – An Overview	120
US Beef Consumption	120
Exhibit 43. Beef Consumption – United States (2002-2012) in Billion Pounds	120
US Beef Production	121
Exhibit 44. Beef Production – United States Commercial Carcass Weight (2002-2012) in Billion Pounds	121
US Beef Exports	122
Exhibit 45. Beef Exports – United States Commercial Carcass Weight (2002-2012) in Million Pounds	122
Exhibit 46. Beef Exports – United States (2002-2012) in US$ Million	123
Canadian Beef Markets	124
Exhibit 47. Beef Products - Canadian Exports (2002-2012) for Live Animal and Beef CAN$ Million	124
Exhibit 48. United States Cattle – Imports from Canada and Other Countries (2002-2012) in Heads	125
Exhibit 49. United States Beef and Veal –Imports from Canada and Other Countries (2002-2012) in Billion Pounds	126
Bison in Demand after BSE Crisis	127
Confirmed BSE Cases in North America	127
1997 Canada Feed Ban	127
Prevalence of BSE in Canada	128
U$39.8 Million to Alberta for New Feed Regulation Implementation	129
New Regulations for Animal Feed in Canada	129
Beef Industry in Canada Granted Class Action Suit of Compensation	129
Safer Designation for Canadian Cattle from World Organization for Animal Health	130
New Network of Excellence Centers to Study Prion Diseases	130
Control Measures to Prevent Spread of BSE	130
Control Measures to Prevent Transmission of BSE to Human	132
Canada Amends BSE Infection Control Regulations for US Imports	132
Exhibit 50. BSE Cases Diagnosed in North America (1993-2012)	133
3.23 Prions – Market Outlook	134
4. PRODUCT /TECHNOLOGY RESEARCH	135
4.1 Introduction	135
Introduction	135
History	135
Prions	137
Prions Formation	137
Structure and Properties of Prion Proteins	138
Prion Protein Diversity	138
Isoforms	139
PrPC	139
PrPSc	140
PrP’s Role in Long-Term Memory	140
Characteristics of Prions	140
Exhibit 51. Comparison of General Characteristics of Classic Virus and Prions	141
Prion Biosynthesis	141
Prions Replication	141
Prion Strains	142
Species Barrier	142
Cancer and Prions	143
Prion Vaccines	143
Prion Disease Prevention through Vaccine in Mice	143
Vaccination	144
Promising Vaccines against Prion Disease	145
Toxic clumps	145
Gut Reaction	146
Approach to Prevent and Control the Spread of Prion Diseases in Human Beings	146
Methods to Control the Cause of BSE and Scrapies	147
Methods for Controlling the Transmission of Prions via Human Plasma Derivatives	148
Diseases Caused by Prions	149
Exhibit 52. Diseases caused by Prions in Animals	149
Transmissible Spongiform Encephalopathy (TSE)	150
Exhibit 53. List of Prion Diseases and Pathogenesis in Humans	150
Exhibit 54. List of Prion Disease and Pathogenesis in Animals	151
Characteristics of TSE	152
Genetics	153
Epidemiology	153
Research to Treat Prion Diseases	153
Mechanism of Conversion of PrPC to PrPSc	154
Transmission and Causes of Prion Diseases	155
Prion Transmission and Propagation Modes	155
Metallic Poisoning	156
Viral Possibilities	157
Sterilization	157
Prions in Yeast and Other Fungi	157
Mechanism of the Prion Diseases	158
Unsolved Puzzle	158
Effectiveness of Quinacrine for Treating Creutzfeldt-Jakob Disease	159
Analysis of Pentosan Polysulphate for Treating Prion Diseases	159
Difficulty Posed by Blood Brain Barrier	160
Risks Associated with PPS	160
Efficacy Evaluation of Intraventricular PPS Administration	160
Species Barriers for Prions	161
Efforts to Control TSE Related Diseases	162
Effect of TSE Related Diseases on Biopharmaceutical Industry	162
Development of In Vitro Diagnostics Polyclonals Free from Pathogen of Scrapie and BSE	163
Genotype	164
Extensive Monitoring of Disease Pathogens	164
Isolation of the countries based on the extent of cases reported:	164
Approved Monitoring Programme:	164
Conclusion	164
Bovine Spongiform Encephalopathy (BSE)	164
Source of the Epidemic	166
Causes	166
Cases of BSE	166
Measures to Prevent the Spread of BSE	167
BSE Testing	167
Ante-Mortem Assays	168
Regulations	168
BSE/TSEs Testing Regulations Imposed by European Union	169
Transmissible Mink Encephalopathy (TME)	170
TME Related Diseases	170
Clinical Characteristics of TME Infected Animals	171
Diagnosis	171
Prevalence of the Disease	171
Stetson Ville Outbreak	172
Scrapie	172
History	172
Introduction	173
Epidemiology and Transmission	173
Signs & Symptoms	174
Wool Manufacturing Process – An Effective Route of BSE Propagation	174
Research	175
Diagnosis of Scrapie	176
Immunohistochemical (IHC) Detection	177
Procedures and Techniques of Immunoblotting	179
Capillary Electrophoresis Immunoassay	179
Evaluation of tests for TSEs by European Union (EU)	180
Eradication Program	180
Exhibit 55. Number of Confirmed Scrapie Cases Reported in Great Britain (1993-2014)	180
Scrapie Cases Reported	181
Feline Spongiform Encephalopathy (FSE)	182
Cause	182
Common Breeding Age and Occurrence	182
Common Symptoms	182
Diagnosis	183
Treatment	183
Prognosis	183
Research	183
Chronic Wasting Disease (CWD)	183
History	184
Major Consequences of CWD Prevalence	184
Causative Agent	185
Clinical Duration	185
Characteristics of Chronic Wasting Disease	185
Diagnosis	186
Development of Aptamer Beacons for Ante-Mortem Diagnosis of Chronic Wasting Disease	186
Epidemiology	187
Distribution	188
Prevention & Control Strategies	188
Kuru	189
Introduction	189
Transmission	190
Diagnosis	190
Treatment	190
Gerstmann-Sträussler-Scheinker Syndrome (GSS)	190
Cause	191
Symptoms	191
Prognosis	191
Creutzfeldt - Jakob Disease	191
Symptoms of CJD	193
Transmission of CJD	193
Precautions to Control Spreading of the Disease	194
Research	195
CJD-Types and Characteristics	195
Sporadic CJD	195
Diagnosis	195
EEG (Electroencephalography)	196
Cerebrospinal Fluid 14-3-3 Analysis	196
Magnetic Resonance Imaging	197
Variant CJD (vCJD)	197
Definition	198
Origin of vCJD	198
Differences between spCJD and vCJD	198
Differences between Classic and Variant CJD	199
Prevalence of vCJD	200
Diagnosis	201
Magnetic Resonance Imaging	201
Tonsil Biopsy	202
CSF 14-3-3	202
Molecular Diagnosis of vCJD through Ante-Mortem Tonsil Biopsy	202
Genetic CJD	203
Diagnosis	203
Iatrogenic CJD	203
Diagnosis	203
EEG (Electroencephalography)	205
Biomarkers	205
14-3-3 Protein	205
Sensitivity and Specificity of 14-3-3 Protein Vary with the form of CJD	205
Metallothionein	206
Laminin Receptor Protein / Laminin	206
Exhibit 56. Worldwide Number of Reported Cases of vCJD by Country (2012)	206
Exhibit 57. Referrals of Suspect CJD, Deaths of Definite and Probable CJD in United Kingdom	207
CJD Control Measures in United Kingdom and United States	208
Centers for Disease Control and Prevention	208
Treatment	208
Fatal Familial Insomnia (FFI)	209
History	209
Pathophysiology of Disease	209
Disease Progression	209
Treatment	210
Common Symptoms and Time Duration Observed in FFI	210
Diagnosis	210
Prions Detection Technologies	210
Immunocapillary Electrophoresis	211
Principle of ICE	211
Sample Source	211
ICE Methodology	211
Parameters	212
Sensitivity of ICE (Immunocapillary Electrophoresis)	212
Limitations and the Present Status	212
Heartbeat Clue to Diagnosing vCJD	214
Conformation Dependent Immunoassay (CDI)	215
Merits of CDI	215
Sensitivity	215
Quantification	215
Rapid Screening	216
Early Diagnosis	216
Other Benefits	216
Immunoblot (IB)	217
Immunoblot Analysis	217
Histopathology and Immunohistochemistry (IHC)	218
Immunohistochemistry	218
Protein Misfolding Cyclic Amplification (PMCA)	219
The PMCA Assay	220
Amplification of rPrPC to rPrPSc in vitro	220
Amplification Technique Improves Prospects for Diagnostic Tests	220
Western Blot Assay for TSE	221
Electrophoretic Pattern of PrP	222
Western Blot Assay for PrP	222
Synthetic Ligands	223
A Protein-Binding Ligand System for Prion Disease Detection	223
Blood Testing for the Prion Disease Agent	224
Immuno-PCR	225
Biomarkers for Detection of Prions (Surrogate Markers)	226
Prion Infection	226
Surrogate Markers	227
Other Assays for Diagnosis of Prion Diseases	228
Platelia Test (ELISA, BioRad)	228
Detection	228
Enfer test (ELISA, Enfer)	228
Prionics-Check LIA (ELISA, Prionics AG)	228
CDI (Inpro)	228
Histoblot	229
Paraffin-Embedded Tissue (PET) Blot	229
Genotyping PrP	229
ELISA and DELFIA	230
Rapid Assay	230
Advantages of this method	230
RNA Aptamers and Prion Proteins	230
Prion Protein Binding RNA Aptamer	230
Interaction of RNA Aptamers with PrPc	231
RNA Aptamer as a Diagnostic and Therapeutic Agent	231
Aptamer and Mutations	231
Animal Bioassays and Cell Culture Assays	232
Mechanism of Prion Formation	232
Animal Bioassays	233
Cell Culture Bioassays	233
Genetic Engineering Tools	233
Prion Decontamination and Inactivation Techniques	234
General Methods of Decontamination	235
Facility Issues	235
Device Issues	236
Novel Methods to Restrict Decontamination of Surgical Instruments	237
Enzyme-Detergent Method to Sterilize Prions	237
Latest Methodologies Restricting the Infections Caused by Prion-Contaminated Medical Appliances	237
Available Alternatives or Methodologies	238
Conditions Normally Used for Inactivating Prions	238
Conclusion	238
Analysis	238
Clearance Studies	238
Prion Clearance Studies	239
Prion Inactivation and Removal	240
Creutzfeldt - Jakob Disease	240
Bovine Components	240
Sanitization of Prion	240
EMEA Clearance	241
Effective Mode Prion Clearance	241
The Regulatory Environment	241
The Nature of the Agent	242
Spiking Agents	242
Yardsticks for Clearance	242
Investigative Steps	243
Inactivation Techniques	243
Exhibit 58. Ineffective or Unreliable Methods of Eradicating Prions	244
Conventional Methods for Inactivation of Prions	245
Use of NaOH and NaOCl for Chemical Disinfection	246
Techniques for Inactivation of Prions in Reusable Instruments and Surfaces	247
Application of Maillard Reaction to Minimize Prion Activation	248
ViruSure	248
Evaluation Protocol	248
Evaluation Process	250
Criteria for Submission of Dossier	250
Pre-Evaluation Assessment	251
Criteria for Admission to Full Evaluation	252
Prion Reduction Filters	252
Core Concerns	252
TSE Transmission via Blood	252
Filters to Facilitate Reduction in Prion Proteins	253
The Effectiveness of the Pall Corporation Filter	253
UKBS Approach to Filters	253
Experimental Models for Endogenous Infectivity in Blood	255
Donor Species	255
Prion Strain	255
Method to Detect Infectivity/PrPSc	256
Advice Sought From the Committee	256
Post Mortem BSE Testing	256
IDEXX HerdChek® Bovine Spongiform Encephalopathy Antigen Test Kit EIA	257
Description and Principles	257
Parts of the Kit	258
Sampling and Preparation	258
Step 1	258
Step 2	259
Step 3	259
Step 4	259
Preparation of Reagents	259
Wash Solutions 1 and 2	259
Plate Diluent Component 2	259
Working Plate Diluent	259
Negative and Positive Controls	260
HRPO-Conjugated Anti-PrP Antibody Solution	260
Acid Stop Solution	260
Test Procedure	260
Assay Protocols	260
Sample Dilution in Working Plate Diluent	260
Use of Plate Shaker for Sample Incubation Protocols – Short and Ultra-short	261
Results	261
Precautions	261
Safety Information	262
Enfer TSE assay	262
Principle	262
Buffers	262
Merits	263
Prionics®-Check WESTERN	263
Method	263
Merits	263
Prionics®-Check LIA	263
Method	263
Merits	264
Prionics®-Check PrioSTRIP	264
Method	264
Merits	264
Post Mortem CWD Diagnosis	265
Antigen-Capture Enzyme-Linked Immunoassay	265
Principle	265
Protocol of EIA	265
Merits of EIA	266
Efficacy of EIA	266
Prions Risk Assessment	268
Measuring Risks Related to Prions	268
Prion Diseases Management Policies	269
Health Control Policies in UK	269
European Union and BSE controls	269
Exhibit 59. EU Preventing the Introduction and Spread of BSE	270
Japan and BSE	270
FDA’s Qualitative Risk Assessment	271
Modes for Transmission of Prions through Cosmetics	271
Identification of Hazard	272
Dose-Response Assessment	273
Characterization of Risk	274
Inferences	274
Guidance on Minimizing the Risk of Transmitting Animal Spongiform Encephalopathies via Human and Veterinary Medicinal Products	275
Regulatory Compliance	275
Risk Assessment	275
Legal Aspects	275
Clarification	276
Scope of the Note for Guidance	276
Relevant Animal Species	276
Materials	276
Seed lots, Cell Banks and Routine Fermentation/Production	277
General Considerations	278
Scientific Principles for Minimizing Risk	278
Source Animals	278
Geographical Sourcing	278
Bovine Materials	278
European Commission Scientific Steering Committee Classification	279
Sheep and Goats (Small Ruminants)	279
BSE Negligible Risk (Closed) Bovine Herds	280
Parts of Animal Bodies, Body Fluids and Secretions as Starting Materials	280
Age of Animals	282
Manufacturing Process	282
Cleaning Process	282
Validation	283
Risk/Benefit Evaluation	283
Specific Considerations	284
Collagen	284
Structural Protein	284
Function of Collagen in the Human Body	284
Safety Measures Offered by Collagen	285
Clinical Perseverance of Collagen Product	285
Enhanced Performance	285
Superior Flexibility	285
Products Derived from Collagen and its Multiple Biomedical Applications	285
Procedure Followed in the Extraction of Collagen	286
Properties and Characteristics of Atelocollagen	286
Additional Characteristics of Atelocollagen	286
Processing of Atelocollagen to Desired Configurations	286
Mutable Characteristics of Available Atelocollagen	287
Superior Quality of KOKEN Atelocollagen	287
KOKEN Atelocollagen is Absolutely Safe	287
Application of Collagen in Various Biomedical Applications	287
Application in Clinical Medicine	287
Tissue Culture Research	287
Cosmetics	287
KOKEN Special-Purpose Atelocollagen Design	288
Gelatin	288
The source material that has been used.	288
Manufacturing methods	288
Bovine Blood Derivatives	289
Traceability	289
Geographical origin	289
Stunning Methods	290
Tallow Derivatives	290
Animal Charcoal	290
Milk and Milk Derivatives	291
Wool Derivatives	291
Amino Acids	292
Product Trends	292
A New Fluorescent Molecule Effective in Diagnosing Prion Disease	292
New Test to Detect Bovine Spongiform Encephalopathy	292
Hematech – Company Develops New BSE Resistant Cow	293
Recent Progress in Prion Research	294
Blood Test to Find Fatal variant Creutzfeldt–Jakob disease	294
Prion like Proteins Helps to Create Long-Term Memories	294
Understanding the Life of Prion Molecules	295
Shape of Infectious Prions	296
Creutzfeldt–Jakob Disease can be Transmitted by Blood	297
1 in 2,000 People in UK Carry Variant CJD Proteins	298
Recombinant Human Prion Protein	299
Prion like Proteins Dispel many Diseases of Aging	299
New Models developed to further the study of Human Prion Diseases	300
Role of HSPG Receptors in the Spread of Misfolded Proteins	301
Ribosomes New Target for Antiprion Medicines	301
New Method to Protect Prion Protein from Changing Shape and Becoming Infectious	302
Flexible Tail of Prion Protein found to Damage Brain Cells	303
A likely Treatment for Prion Disease in Humans	303
CJD May be Manifested as Rapid Hearing Loss	304
Iron Metabolism Controlled by Normal Prion Protein	305
More Disease Causing Proteins in Brain Disorders	306
Protective Prion Stimulates Transition in Yeast Cells	307
Adaptation of Infectious Prions to Multiple Hosts	308
Extracts from Green Tea and Red Wine can disrupt Alzheimer’s Disease Pathway in Cells	309
Role of Normal Prion Protein in Brain Development	310
Mode of Transmission of Infectious Prion Proteins	310
US$13.5 million Neurodegenerative Biomarker Consortium to be Lead by Karolinska	311
Potential Biomarker for Creutzfeldt-Jakob Disease Discovered by Case Western University	311
Transmission of Prions through Aerosols	311
Early Diagnosis of Prion Diseases: NIH	312
New Prion Discovery Shows Drug Target for Mad Cow Disease and other such Illnesses	313
Role of Aggregate Size in the Spread of Prion based Disease	314
Discovery of Variably Protease-Sensitive Prionopathy (VPSPr) and its Relation with Creutzfeldt-Jakob disease (CJD)	315
Possible Transmission of CJD through Surgery	317
In Normal Brain Tissue Infectious Prions were Found to Come up Unexpectedly	317
Vaccines for Prion Diseases	318
Role of the Sequence and Structure for Prion Disease Transmission to New Hosts	319
Cattle Eye – Indicator of Mad Cow Disease	319
Additional BSE Testing in Cattle by Alberta	320
Prion Proteins and Cellular IPOD	320
Occurrence of a New Form of Prion Disease	321
Synthetic Recombinant Infectious Prion Protein	322
Prion Leaves Sustainable Impression on Memory	323
Prion Protein in Cell Culture	324
Novel, Fast-Acting Disinfectant against Viruses, Bacteria, Fungi and Prions could help Reduce the Spread of Lethal Infections in Hospitals	325
Transformation of Normal Prion Protein into Infectious Form by Misfolding	326
Prions and its Role in Causing Evolutionary Changes, Adaptation and Diseases	327
New Protein that is Vital to Development of Bovine Spongiform Encephalopathy (BSE) Discovered	329
Evolvement of a Brain Disease Resistance Gene in a Community in Papua New Guinea may Provide Insight into CJD	329
Prion Disease and Cholesterol Biosynthesis	330
Molecular Structure of Misfolded Prion Proteins	330
Cell Study explains why Younger People are More Prone to vCJD	331
Detection of vCJD Prions from Non-Human Primates by Amorfix	332
Mutation in Gene-Enough to Cause Contagious Prion Disease	333
Impaired Transport in Neurons Trigger Prion Disease	334
Discovery of the method adopted by prion proteins in Brain Cell Damage could help in Better Comprehending Mad Cow Disease and Related Disorders	335
Socioeconomic Aspects of CWD in Canada	337
Link Between Copper and Functioning of Prions Related to BSE & CJD Discovered….	338
Prions and Phenotypic Diversity	339
Role of Iron in Prion Disease- Associated Neuronal Demise	340
Treatment of Variant Creutzfeldt-Jakob Disease (vCJD) with Antibody	340
Prions and Adaptive Gene Expression	341
Susceptibility of Prions to Birnessite	342
Presence of Prions in White And Brown Fat Tissues of Mice	342
Role of Mutant Proteins in Infectious Prion Disease in Mice	343
Mouse Model of Prion Disease Mirrors the Symptoms of Human Disorder	343
First Constructed Prion Protein with an Anchor	344
Alternate Pathway not very Useful for Prions	345
Unraveling the Mystery behind Prion Proteins	345
Infectious, Test Tube-Produced Prions Can Jump the ‘Species Barrier’	346
The Findings on a Research Study on Animals Reveal that a Variation of Mad Cow Disease may be Transmitted through Blood Transfusions.	348
Tracking Prions	349
Prions show their Good Side	350
Scrapie Prions from Asymptomatic Sheep by Amorfix	350
Resistant Prions	350
Transmission of Prion Diseases in Milk	351
Prions Associate with Cholesterol to Cause Neuro-Degeneration….	352
Glowing Molecules to Detect Prions…..	353
Prions May Activate Retroviruses in Infected Brain Cells	353
Small Clumps of Prion Proteins May be Primary Cause of Neurodegenerative Diseases	354
Newly Discovered Prion Proteins May Offer Insight into Mad Cow Disease	355
Prion Proteins Discovered to Prevent Development of Alzheimer’s Disease	356
Manganese Levels Mount in Scrapie-Infected Sheep Prior to Development of Clinical Symptoms	357
Vaccine Prevents Prion Disease in Mice	357
Safe Disposal of Prion Diseases Infected Animals…..	358
Resin Beads Developed to Seize Mad Cow Disease Agent through Blood	358
Single Prion Molecules’ Structural Dynamics	359
Prion Free Genetically Modified Cattle…	360
V. CORPORATE DIRECTORY	361
VI. PATENTS INFORMATION	377

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