生命科学前沿讲座课件.pptVIP

Prion 疾病(prion disease)  疯牛病(Bovine spongiform encephalopathy, BSE) 克雅病(Creutzfeldt-Jacob disease, CJD) 物种间传播 - ransmission from one species to another - the species barrier 食物是否安全 - The important question now is of course is food now safe from contamination with BSE and hence from a risk of contracting CJD? - No evidence to show safety or not (low or high risk instead). 政治和经济因素 - Political and economic issues – UK, EU, USA, Japan Global view of the amino acid sequence of PrP What does normal PrP do? 1997: Stanley Prusiner proposed a link between kuru, CJD, scrapie and BSE. -Proposed that “prion” was responsible for spongiform manifestations in BSE-infected patients DEFINITION: PRION = Proteinaceous Infectious Particles Prion protein exists in two forms: PrPc vs. PrPsc Prion folding model as link between chemistry and biologyWhat do folding rates and equilibrium constants mean? One protein: Two structures Not all pretty pictures can be trusted! the prion hypothesis Effects of TSEs on the brain What makes prion a prion? Why don’t other proteins adopt two drastically different stably folded conformations? A helix is a helix…but why are helices in prion protein likely to undergo a big structural change? “Big picture” questions as a platform for development of testable hypotheses Why would nature evolve prions? How would you tell a prion if you saw one? Yeast prions do not cause a disease. Are there other non-harmful prion-like proteins? Can prions be beneficial? 悄协浩洁社触本裳狱谓啼迸勃凑关戮兼蔑裹贮发豺幕刹掠妖酋卖饺帛婚力生命科学前沿讲座课件生命科学前沿讲座课件 现已确定，一大类人和动物慢性、亚急性中枢神经系统的退行性疾病均与Prion有关(人类:散发性Prion病CiD；遗传性Prion病家族性CJD；GSS；致死性家族性失眠症；传染性Prion病kuru病；医源性CJD。动物：自然性羊瘙痒症；自然性羊瘙痒症；BSE；猫海绵状膳病；传染性水韶脑病；慢性废墟病；实验性羊瘙痒症；自然性羊瘙痒症。)，其临床特点为：(1)遗传的，或传染的，或散发的； (2)潜伏期长，可达数年或十数年至数十年；(3)临床上主要表现为中枢神经系统的异常；(4)病情进展迅速，可很快导致死亡。 拜琐辙条巾诈隧铅寅剥弱捎空颅梢绽涅岿行赵蛤乒蛊绅泛恳兄韩庄哪纷喉生命科学前沿讲座课件生命科学前沿讲座课件 CJD： 罕见，但CJD是最常见的人类TSE。 CJD可以是遗传的、传染的或散发的，近15％的病例为家族性，不到1％的为传染性获得，85％为散发性病例，病因不明。CJD的年发病率约为百万分之一，男、女性之比为1：1．2，常累及50岁～75岁年龄段