Parchi P, Gambetti P
Division of Neuropathology, Case Western Reserve University, Cleveland, Ohio, USA.
Curr Opin Neurol. 1995 Aug;8(4):286-93. doi: 10.1097/00019052-199508000-00007.
Major advances have been made in prion diseases. Recent data indicate that the prion protein is likely to be a synaptic protein with a functional role in synaptic transmission. An impressive body of evidence suggests that (1) the normal prion protein plays a central role in prion replication; (2) the replication process implies an interaction between the normal prion protein and the pathogenic prion protein; and (3) the pathogenic prion protein is the infectious agent, the infectivity of which is dependent on its abnormal conformation. Genetic and protein analyses have expanded the spectrum of prion diseases and have underlined the complexity of genotype-phenotype interactions.
朊病毒疾病已取得重大进展。最近的数据表明，朊病毒蛋白可能是一种在突触传递中具有功能作用的突触蛋白。大量令人印象深刻的证据表明：（1）正常朊病毒蛋白在朊病毒复制中起核心作用；（2）复制过程意味着正常朊病毒蛋白与致病性朊病毒蛋白之间存在相互作用；（3）致病性朊病毒蛋白是感染因子，其感染性取决于其异常构象。遗传和蛋白质分析拓宽了朊病毒疾病的范围，并突出了基因型 - 表型相互作用的复杂性。
