因 cD From Dr Jekyll to Mr Hyde Prpe The prion protein exists in two forms.The normal, innocuous protein(PrPc)can change its shape to a harmful,disease-causing form(PrPSc).The conversion from PrPc to PrPSc then proceeds via a chain-reaction.When enough PrPSc proteins have been made they form long filamentous aggregates Prps that gradually damage neuronal tissue.The harmful PrPSc form is very resistant to high temperatures, UV-irradiation and strong degradative enzymes
回 Prions infect animals and humans 羊搔痒症(scrapie) All known prion diseases are fatal.Since the immune system does not recognize prions as foreign,no natural protection develops.Scrapie in sheep was first described during the18th century.It has been transmitted to other animals such as mink and cats, and more recently to cows(mad cow disease or bovine spongiform encephalopathy,BSE)through contaminated feedstuff 牛海绵状脑病 (spongiform encephalopathy) In New Guinea,the Fore- people contracted kuru by eating the brains of deceased people Creutzfeldt-Jakob Disease (CJD)froquontl arises spontan while fatal fam insomnia(FFI) 人的库鲁病(kuru)、克雅氏病 Gerstmann-Str Scheinker (GSS (Creutzfeldt Jakob disease,CJD) and 10-15%of caused by mutations in the gene encoding the prion protein.A new variant CJD,diagnosed in some 20 patients,may have arisen through transmission of BSE to humans
人的库鲁病(kuru)、克雅氏病 (Creutzfeldt Jakob disease, CJD)等 羊搔痒症(scrapie) 牛海绵状脑病 (spongiform encephalopathy)
NOBELFORSAMLINGEN KAROLINSKA INSTITUTET har beslutat att 1997ars NOBELPRIS ifysiologi eller medicin skall tillerkanas Atdem somhiargiort manskligheten den storsta ntta StanleyB.Prasiner Nobels testcmente for hans upplackt ad prioner ○ Ra2 Prusiner(1982)提出羊柽痒病因子是一种蛋 白质侵染颗粒，并将之称做Prion.或Virino。 一朊病毒 1997年，Stanley B.Prusiner荣获诺贝尔奖
Prusiner (1982)提出羊搔痒病因子是一种蛋 白质侵染颗粒,并将之称做Prion或Virino。 -朊病毒 1997年，Stanley B. Prusiner荣获诺贝尔奖
三、朊病毒的发现与思考 1)蛋白质可以作为遗传物质？ prion,是生命的一个特例？ 表达调控的一种形式？ 2)蛋白质折叠与功能的关条，是否 存在折叠密码？
三、朊病毒的发现与思考 1）蛋白质可以作为遗传物质？ prion是生命的一个特例？ 表达调控的一种形式？ 2）蛋白质折叠与功能的关系，是否 存在折叠密码？