Creutzfeldt Jakob. PrP Sc PrP Sc 10

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1 Creutzfeldt-Jakob CJD BSE CJD Creutzfeldt-Jakob CJD PrP c PrP Sc PrP Sc 10 PrP c PrP Sc BSE 11 BSE CJD BSE 3 CJD BSE Creutzfeldt-Jakob Creutzfeldt-Jakob Creutzfeldt-Jakob CJD Creutzfeldt Jakob bovine spongiform encephalopathy BSE CJD Creutzfeldt-Jakob CJD variant CJD Stanley B. Prusiner Proteinaceous infectious particle Prion prion protein PrP 1253 PrP c Prusiner 1997

2 BSE scrapietransmissible mink encephalopathy PrP Sc Gajdusek 1977 CJD Periodic Synclonous Discharge PSD 3 4 PrP Sc BSE CJD 2CJD 3 Gerstmann-Straussler-Scheinker GSS 4 5 6CJD 7CJD Creutzfeldt-Jakob vcjd

3 CJD BSE vcjd vcjd 1996 Lancet A new variant of Creutzfeldt- Jakob Disease (vcjd) CJD Val 129 Met 3 MRI 5 PrP c PrP Sc

4 CJD 7Val 129 Met 4nm nm PrP Sc PrP Sc PrP c PrPPrP BSE BSE vcjd BSE PrP c PrP Sc PrP Sc PrPPrP 30 CJDvCJD

5 CJD BSE Val 129 Met 4 PrP PrP c PrP Sc vcjd Prusiner PrP Sc 8 vcjd Eigen 10 PrP Sc nm nm PrP Sc PrP Sc 1996CJD CJD 747 CJD 82vCJD BSE BSE BSE

6 vcjd BSE 13 Control and Prevention CDC vcjd 22 CDC vcjd CJD BSE CDC BSE Centers for Disease BSE

7 CJD BSE CJDCJD PrP Sc PrP Sc BSL-2 3 SDS sodium dodecyl sulfate PrP Sc CJD CJD vcjd hgh-cjd M M M V V V Met Val vcjdcjd GH-CJDCJD 1 3 SDS

8 BSE PrP CJD 12 BSE vcjd vcjd BSE vcjd Sky/2572/klink.html 01 Prusiner SB. Prions. Proc Natl Acad Sci USA 1998; 95: Will RG, Ironside JW, Zeidler M, et al. A new variant of Creutzfeldt-Jakob disease in the UK. Lancet 1996; 347: Tateishi J, Ohta M, Koga M, et al. Transmission of chronic spongiform encephalopathy with kuru plaques from humans to small rodents. Ann Neurol 1979; 5: ; 122: ; 58: Creutzfeldt-Jacob 2003; 58: Zeidler M, Sellar RJ, Collie DA, et al. The pulvinar sign on magnetic resonance imaging in variant Creutzfeldt-Jakob disease. Lancet 2000; 355: Brown P, Will RG, Bradley R, et al. Bovine spongiform encephalopathy and variant Creutzfeldt-Jakob disease: background, evolution, and current concerns. Emerg Infect Dis 2001; 7: Osherovich LZ, Cox BS, Tuite MF, et al. Dissection and design of yeast prions. PLoS Biol 2004; 2: Eigen M 2002; Jan: ; 43: ; 43: Llewelyn CA, Hewitt PE, Knight RS, et al. Possible transmission of variant Creutzfeldt- Jakob disease by blood transfusion. Lancet 2004; 363: Hillier CE, Salmon RL, Neal JW, et al. Possible underascertainment of variant Creutzfeldt- Jakob disease: a systematic study. J Neurol Neurosurg Psychiatry 2002; 72:

9 CJD BSE Variant CJD and BSE Masanori Nakagawa Research Institute for Neurological Diseases and Geriatrics, Department of Neurology and Gerontology, Kyoto Prefectural University of Medicine Graduate School of Medical Sciences Bovine spongiform encephalopathy (BSE), also known as "mad cow disease," is a fatal brain disease of cattle first recognized in the United Kingdom. In humans, the most common transmissible spongiform encephalopathy is Creutzfeldt-Jacob Disease (CJD). Both diseases are classified into Prion disease. Although no cases of CJD have been directly linked to beef consumption, an advisory committee of the United Kingdom has reported that recent cases of a variant CJD (vcjd) may be associated with BSE in This announcement has alarmed consumers well beyond the borders of the United Kingdom. vcjd is characterized by young age at onset, early psychiatric symptoms, prominent ataxia, absence of periodic electroencephalographic activity, a comparatively prolonged illness, and plaque formation in the brain. It is still unknown how normal human prion proteins (PrP c ) are converted to abnormal prion protein by abnormal bovine prion proteins (PrP Sc ). It is suggested that the aggregated prionic form is most probably a necessary, but possibly not sufficient, prerequisite of infection. Eleven cases of BSE have been reported in Japan, but the beef was not distributed to the Japanese consumers because of Japanese safety system for BSE. No vcjd has been reported in Japan. The first BSE has been recognized in the United States last December. Japanese government immediately prohibited the import of beef from the United State. No sufficient therapies have been established, but pentose sulfate may have some effects for vcjd. : Creutzfeldt-Jacob disease, Variant Creutzfeldt-Jacob disease, Bovine spongiform encephalopathy, Mad cow disease, Prion

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