1976年诺贝尔生理医学奖—蛋白粒子类疾病.ppt

1976年诺贝尔生理医学奖——蛋白粒子类疾病 制作者——晏婷婷 生物工程学院03级1班 丹尼尔·卡里顿·盖达塞克D.Carleton Gajdusek 1923年出生于美国 哈佛大学毕业 由于查明库鲁病病因而获得诺贝尔奖 “如果你喜欢到未开化的地区去探险，那么，你有可能获诺贝尔奖。” 库鲁病——kuru(also known as laughing sickness due to the outbursts of laughter that mark its second phase ) Kuru was first noted in New Guinea in the early 1900s. Symptoms of kuru:regularly progress to neurological degeneration in three to six months (usually three) and to death is another matter and cannot be shrugged off. 患有库鲁病的弗雷族小孩（如右图） kuru Another characteristic finding of kuru is shown on this slide: brush-like plaques, which are also called spikeballs and found in the cerebellum. ? ?Misinterpretations of kuru Scholars who first studied the disease had major misconceptions concerning its nature. They first incorrectly postulated that it was a genetic disorder, as it had a tendency to occur among family members. This possibility was eventually ruled out because kuru was too common and too fatal — such a lethal genetic disorder would drastically reduce the fitness of a population and soon die out of the gene pool. Gajdusek research on the kuru Gajdusek conducted studies on chimpanzees injected with brain material from a victim. These studies led scientists to hypothesise that the agent was a slow virus, because the chimps developed a very similar condition after a long incubation period. Gadjusek defined a slow virus as a viral disease with an abnormally long incubation period. In humans, kuru had an incubation period with a minimum of two years and maximum of twenty-three (Gajdusek et al., 1966). Later studies showed the slow virus hypothesis to be a misinterpretation as well. Gajdusek’s results however confirmed the infectious, transmissible nature of the prion. The prion protein 正常的朊蛋白位于神经细胞的表面，大约是最小病毒尺寸的1/100。它是折叠为两种形式之一的蛋白质。 朊蛋白导致库鲁病的原因 朊蛋白又称普里昂蛋白，它可加速进化。朊蛋白可能对酵母进化提供了一个加速度。如右图 Prion protein（prp) 朊蛋白以蛋白质为主体，没有核酸存在的病毒。 正常的prp富含α—螺旋，称为prpc PrPc在某种未知蛋白质的作用下可转变成全为β—折叠的PrPsc,从而致病。 盖达塞克的实验 死者的脑子研碎——滤洗(除去微生物）——核酸分解酶处