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2 SRM TMETransmissible Mink Encephalopathy CWDChronic Wasting Disease BSE 1

3 WB IHC BSE SRM SSOPHACCP BSE AMR 2

4 BSE BSE 3

5 4

6 Bovine Spongiform Encephalopathy BSE )2) 3) 4) BSE 5) BSE ) ) BSE 8) 9) SPS 10) 5

7 BSE BSE BSE 3 BSE BSE SRM 3 BSE 12) BSE 4) 1015BSE 13) SRM 20 14) 20 13) SRMBSE ) 524 1) BSE ) SRM 20 16) ) 6

8 17) 18)BSE EC ) ) BSE SRM 20 19) 20) BSE 7

9 BSE SRM SSOPHACCP AMR BSE BSE BSE BSE 450 9, , ) BSE BSE 1, ) 1989 BSE 22) ) BSE

10 23) 117 BSE 23) ,762 23) ) 497 1, , ) ) ) /100 1,711/ ) / /33227/ ) 4 26),27) BSE (6 7)/ ) 1994 BSE 1996 BSE 22) ) ) ) { } ) ) 9

11 { } 16,000340, ) /100291/ { } /33201/ BSE 22) ) ) t 23) 524t 140 ( , ) 227, ,863t 23) ,000t , ,000 25) ,129 / ,000/ ) 1990 BSE 2000 BSE 22) t 2 28) 149t 28) 0t 5,710 11,046t 0 11t 5,699 11,046t 28) 0 11t{ 5,710 5,69911,046 11,046 } 0 26t 10

12 250, ,000t 28) t 0/100 11/ t 1/5, ,245 25) 29) ) t 29) GBR ,000t 2,000t 29) 29) ) t 29) 1/2 1/12 33,000 6,500 10,000 3,000 5,000 25,00012,000 7,200 4,200 1,200 25) BSE 1/10 1/ /12~1/47 5,100 1/2 1/

13 BSE 80 90% 60 21) SRM ) BSE ) 2004SRM 32) FDABSE 33) BSE % ) SRM 35)36) ) BSE 10 38) )

14 BSE ) / ) ,1216,199 43) FDA 44) General Accounting OfficeGAO 45) ) ) ) FDA 12,575 FDA 3, ) GAO FDA BSE 45) CFIA BSE 1997 CFIA 49)

15 ) 50) ) ,129 51) ) 52) SRM 53) 665 1, ) ) SRM BSE 99.4SRM 55)SRM SRM 53) SRM 56) 30 SRM 57) SRM 58) 30 SRM SRM SRM ) FDA ) TMETransmissible Mink Encephalopathy BSE TSE Transmissible Spongiform EncephalopathyTME TME TME 1985 TME TME TME 60)

16 30) TME 61) TME CWDChronic Wasting Disease 1967 mule ) CWD 1% 71 1% 17% 62) CWD BSE 63) CWD CWD BSE )34) BSE ECBSE 8,000 ID50 ID5050% 55) ) SRM 1 P=1 10,000 ID50 OIE /100 64) 100 ID50 10 P= ID P= ID50 10 ID

17 100 BSE ) 1993 (Animal and Plant Health Inspection ServiceAPHIS) National Veterinary Services Laboratories NVSL)IHC Immunohistochemistry 65)66) ,829 67) IHC ,654 67) BSE ) ) ELISA(Enzyme-linked immunosorbent assay) IHC ,477 70) 16

18 2 BSE ) ELISA 72) NVSL State Veterinary Diagnostic LaboratoriesSVDL ) ,467 ELISA 74) 3 IHC (Office of Inspector General OIG) WB Western blot WB (National Animal Disease CenterNADC) Veterinary Laboratories AgencyVLA WB IHC IHC 71) 75) 2004 ELISA WB ELISA WB IHC IHC BSE OIE Manual of Standards WB 76) WB BSE 17

19 1992BSE )78) BSEIHC 79) BSE1 BSE20041ELISA , , ) BSE IHC 79) 20039WB2004 ELISA 79) TSE CFIAWBELISA National Centre for Foreign Animal DiseaseNCFADBSEIHC IHCWB 78)80) 18

20 1) 2) BSE 3) ) ) ) , BSE 81) ) ) ELISA WB IHC 82) 82) ,398 ELISA IHC WB 2005 BSE ELISA BSE BSE 12) ELISA WB IHC BSE 114 ELISA WB IHC 19

21 12) ,974, BSE 83) BSE BSE BSE 20 BSE BSE OIE BSE BSE 2 ELISA WB IHC 84) NVSL Standard operation procedure Appendix 1 (Obex) 72) ELISA 72) 77) 78) EU 3 12) a) WB OIE Manual of Standards WB SAF Immunoblot 76) Proteinase K SAF SAF 20

22 350g WB b) (IHC ) IHC IHC NVSL SOP GPPISOP ) OIE IHC OIE manual 85) ELISA WB IHC 12) IHC 2005 WB ELISA WB IHC IHC EU BSE BSE BSE 86) BSE % ) 9, ) 4,200 3, ) ,100 8, ) 3, ) 2003 BSE

23 BSE BSE 11 3 BSE ) /35 BSE 1 2 / / / /12.3 BSE BSE % 52,817 80, BSE /8 90 1/ / BSE 22

24 BSE(2003) 9,500 4,200 3, , , % 503,3001.5%) % , , % 11, % 86, %) %1 3/ / /380 6/98 5/100 32/3, / / BSE /12 1/47 1/5,100 BSE BSE SRM BSE BSE BSE 100 BSE 23

25 ) )90) 58) ,500 3, ) A50 A ,748 A50 A %439 99% 0.95% 91) 90 2, ,00024, BSE ) 95)

26 96) 90 2,510 2,700 1,650 1, , ) 98) % 25% 58) 99) ) ) 100) ) BSE 20 BSE ) BSE 103) 105) 1 5,000 58)106) 107) 2 1 2, ) BSE 20 BSE 25

27 BSE 18 BSE ) BSE BSE 10 BSE BSE BSE ) BSE ) BSE ) ) ) % 112) 54) ) 80% SRM SRM SRM 30 26

28 SRM 57) 114) SRM 19) 20) SRM 114) 116) BSE GFAP 117) ) ) 116) GFAP SSOPHACCP SSOPSanitation Standard Operating Procedures 118) HACCP Hazard Analysis and Critical Control Point SSOP SSOP HACCP 89)119) SRM SSOP 2 120) SRM Food Safety and Inspection ServiceFSIS SRM , ) 1,036 1,036 HACCP 405 SRM SRM SSOPHACCP SRM 27

29 6 BSE BSE BSE BSE VLA BSE 122) 123) BSE ) 123) BSE BSE 94 BSE 124) 125) BSE BSE BSE BSE BSE BSE BSE AMR ) ) AMR 28

30 SRM 30 AMR AMR 57) AMR 126) BSE SRM BSE 6 127) BSE 3 BSE WB 3 2 IHC 3 1 BSE 128) FSIS ) SRM SRM SSOP 119) 130) A40 A40 A40 20 A40 21 BSE 20 BSE BSE 20 29

31 BSE BSE SRM SSOPHACCP 80 SRM 20 BSE A40 SRM BSE BSE ,

32 BSE BSE BSE SRM AMR HACCPSSOP SRM HACCP SSOP GFAP SSOP 31

33 A BSE BSE SRM

34 SRM SRM BSE BSE BSE 99.4% SRM SRM 21 33

35 , BSE , BSE Canadian Food Inspection AgencyCFIA 11, Summary of the the report of the investigation of Bovine Spongiform Encephalopathy (BSE) in AlbertaCanada BSE WTO 1 Agreement on the application of sanitary and phytosanitary measures SPS BSE BSE WG BSE BSE United States Department of Agriculture 34

36 USDA USDA EV CFIA BSE European Food Safety Authority EFSAWorking group report on the assessment of the Geographical BSE-Risk (GBR) of United States of America Scientific Steering Committee SSC Update of the opinion of the Scientific Steering Committee on the Geographical Risk of Bovine Spongiform Encephalopathy(GBR) BSE BSE BSE-BSE The BSE Inquiry Vol.16 Reference Material 27. Horn Committee s report EFSAWorking group report on the assessment of the Geographical BSE-Risk (GBR) of Canada Title 21 Code of Federal Regulations Sec FDAExpanded Mad Cow Safeguards Announced to Strengthen Existing Firewalls Against BSE Transmission FDA Center for Veterinary MedicineCVM CVM UpdateFDA AND USDA REQUEST COMMENTS AND SCIENTIFIC INFORMATION ON POSSIBLE NEW BSE SAFEGUARDS FDA FDA NewsFDA Proposes Additional Mad Cow Safeguards CFIACFIA Canada s Feed Ban RegulationsCanada Gazette, Part,Vol.131,No.16,p CFIACFIA News ReleaseNew Regulation Proposed For BSE-Related Feed Controls CFIA CFIA News Release Backgrounder on New Regulations Proposed for BSE-Related Feed Controls

37 DEFRA Department for Environment Food and Rural Affairs BSE: Statistics-Confirmed cases of BSE in GB by year of birth where known OIE Office International des Epizooties Geographical Distribution of Countries that reported BSE Confirmed Cases since Avis du Comite d Experts Speciallise sur les ESST sur le suivi de l epizootied ESB en France BSE CVM CVM Update-Update on Feed Enforcement Activities to Limit the Spread of BSE General Accountig OfficeGAOMAD COW DISEASE,GAO FDA s Management of the Feed Ban Has Improved, but Oversight Weaknesses Continue to Limit Program Effectiveness SSCReport on the Assessment of the Geographical BSE-RiskGBRof the UNITED STATES of AMERICA BSE CVM CVM Update-Update on Feed Enforcement Activities to Limit the Spread of BSE CFIAFeed Ban Review SPARKS COMPANIES INC.North American Feed Complex Multi-Client Study

38 BSE 55. SSC Opinion on the Scientific Steering Committee on the human exposure riskhervia food with respect to BSE Federal Register/Vol.69,No (B)(1) The Secretary s Foreign Animal and Poultry Disease Advisory Committee s SubcommitteeReport on Measure Relating to BSE in the United States BSE BSE Williams, E.S., Miller, M.W. Transmissible spongiform encephalopathies in non domestic animals; origin transmission and risk factors. Rev sci tech Off Int Epiz.(2003) 22: Miller, M.W., Williams, E.S., McTarty, C.W., Spraker, T.R., Kreeger, T.J., Larsen, C.T., Thorne, E.T. Epizootiology of chronic wasting disease in free-ranging cervids in Colorado and Wyoming. J Wildl Dis. (2000)36: Williams, E.S. Chronic wasting disease. Vet Pathol. (2005) 42: Schreuder, B.E.C., Geertsma, R.E., van Keulen, L.J.M., van Asten, J.A.A.M., Enthoven, p., Oberthur, R.C., de Koeijer, A.A., Osterhaus, A.D.M.E., Studies on the efficacy of hyperbaric rendering procedures in inactivating bovine spongiform encephalopathy(bse) and scrapie agents.vet Rec. Vol.142:pp APHIS(Animal and Plant Health Inspection Service)BSE Surveillance ( Harvard Center for Risk AnalysisEvaluation of the Potential for BSE in the United States USDAAdditional Question and Requests to USDA USDASummary of the Epidemiological Findings of North American Bovine Spongiform Encephalopathy Positive Cattle APHISBSE Surveillance Plan

39 ELISAWB IHC 73. NVSL BSE Network Labs APHIS BSE Test Results BSE CFIANational Bovine Spongiform Encephalopathy(BSE) Surveillance program CFIASurveillance Government of CanadaTechnical Overview of BSE in Canada BSE BSE BSE BSE BSE BSE SSOP HACCP CFIAAttachment 3.0 Age Verification CFIAAttachment 3.1 Canadian Cattle Identification 38

40 Agency CFIAAttachment 3.2 ATQ CFIA Attachment 3.3 Audit protocol to verify the accuracy of birth date information in the CCIA and ATQ USDA OVERVIEW OF THE U.S. BEEF INDUSTRY National Agricultural Statistics Service USDA Livestock Slaughter 2004 Summary CFIAOverview of Canada s Safeguards BSE Q&ABSE DEFRA(Department for Environment, Food and Rural Affairs) BSE : Statistics - Youngest and oldest cases by year of onset-gb ( Passive surveillance only ) ( BSE

41 CFIARemoval of Specified Risk Materials(SRM) from Cattle Slaughtered in Establishments Inspected Under the Meat Inspection Regulations, BSE BSE SSOP BSE Wells, G.A., Hawkins, S.A., Green, R.B., Austin, A.R., Dexter, I., Spencer, Y.I., Chaplin, M.J., Stack, M.J., Dawson, M. Preliminary observations on the pathogenesis of experimental bovine spongiform encephalopathy (BSE): an update. Vet Rec. (1998)142: Wells, G.A., Spiropoulos, J., Hawkins, S.A., Ryder, S.J. Pathogenesis of experimental bovine spongiform encephalopathy: preclinical infectivity in tonsil and observations on the distribution of lingual tonsil in slaughtered cattle. Vet Rec. (2005)156: Buschmann, A., Groschup, M.H. Highly bovine spongiform encephalopathy-sensitive transgenic mice confirm the essential restriction of infectivity to the nervous system in clinically diseased cattle. J Infect Dis. (2005) 192: Iwamaru, Y., Okubo, Y., Ikeda, T., Hayashi, H., Imamura, M., Yokoyama, T., Shinagawa, M.: PrPSc distribution of a natural case of bovine spongiform encephalopathy. International Symposium Prion Disease Food and Drug Safety, Sendai, Japan

42 127. SSC Opinion on TSE infectivity distribution in ruminant tissues(state of knowledge,december 2001) BSE FSISTo Allow Use Of Small Intestine From U.S. And Eligible Countries

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1 2 3 4 5 ( ) 6 7 8 "proteinaceous infectious particles" ( ) (PrP Sc ) (PrP C ) PrP C PrP Sc PrP Sc 9 10 (1) (2) 11 12 13 14 15 16 (1) (2) (3) 17 (4) (5) 18 19 20 21 22 23 24 (1) Creutzfeldt-Jakob - -.