Kristensson K
Kliniskt forskningscentrum, Karolinska institutet vid Huddinge sjukhus, Stockholm.
Lakartidningen. 1991 Oct 9;88(41):3387-90.
Recent research on prions and trypanosomes is reviewed as they constitute examples of how infectious agents can cause degenerative diseases or functional disturbances in the nervous system. Prions are novel, transmissible pathogens causing degenerative diseases of the central nervous system both in humans and in animals. Kuru, Creutzfeldt-Jakob's disease and Gerstmann-Sträussler-Scheinker's (GSS) syndrome illustrate the acquired, sporadic and genetic manifestations of the human prion diseases, respectively. Prions are composed largely, if not entirely, of an abnormal isoform of the prion protein, which is a normal cellular protein occurring at high concentrations in the central nervous system. Specific mutations in the prion gene may cause the sporadic and hereditary human disease. S B Prusiner's group at the University of California has used genetic engineering techniques to recreate GSS syndrome by transplanting the mutated prion gene into mice in which the effects of the prion protein genes can then be studied. Trypanosomes are extracellular parasites which cause distinct functional disturbances of the nervous system, although they do not penetrate the blood-brain barrier. Factors are now being isolated in the interactions between the parasites and the immunesystem, and the effects of such factors on the functional properties of neurons are studied.
本文综述了近期有关朊病毒和锥虫的研究,因为它们是传染性病原体如何导致神经系统退行性疾病或功能障碍的实例。朊病毒是一种新型的、可传播的病原体,可导致人类和动物的中枢神经系统退行性疾病。库鲁病、克雅氏病和格斯特曼-施特劳斯勒-谢克尔综合征分别说明了人类朊病毒疾病的后天性、散发性和遗传性表现。朊病毒主要(如果不是完全)由朊病毒蛋白的异常异构体组成,朊病毒蛋白是一种正常的细胞蛋白,在中枢神经系统中大量存在。朊病毒基因的特定突变可能导致散发性和遗传性人类疾病。加利福尼亚大学的S·B·普鲁塞纳团队利用基因工程技术,通过将突变的朊病毒基因移植到小鼠体内来重现格斯特曼-施特劳斯勒-谢克尔综合征,进而研究朊病毒蛋白基因在小鼠体内的作用。锥虫是细胞外寄生虫,虽然它们不会穿透血脑屏障,但会引起明显的神经系统功能障碍。目前正在分离寄生虫与免疫系统之间相互作用的因素,并研究这些因素对神经元功能特性的影响。