遗传和感染朊病毒疾病。
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Prusiner某人
遗传和感染朊病毒疾病。
拱神经。1993年11月,50(11):1129 - 53年。
- PubMed ID
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8105771 (在PubMed]
- 文摘
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丰富分数从叙利亚仓鼠(SHa)大脑对痒病朊病毒传染性导致朊蛋白的发现(PrP)。朊病毒疾病包括痒病牛羊和牛海绵状脑病的克雅氏病(CJD)和Gerstmann-Straussler-Scheinker综合症(GSS)的人类。转基因小鼠(Tg)表达沙和鼠标(Mo) PrP基因被用来探测物种屏障的分子基础和痒病朊病毒复制的机制。生物的大脑提取来自两个scrapie-infected Tg线表明朊病毒培养液确定朊病毒合成新创,即使PrP基因细胞表达。研究人工朊病毒产生嵌合体Mo / SHaPrP转基因强调接种朊病毒的概念规定朊病毒将被复制。发现人类的PrP基因突变的GSS和家庭建立库贾氏症朊病毒疾病都是遗传和感染。转基因小鼠表达高水平的MoPrP-P101L, GSS对应点突变(P102L)在人类PrP,自发发展神经功能障碍,海绵状变性和星形神经胶质过多症。接种的大脑提取准备从这些Tg (MoPrP-P101L)小鼠产生在收件人动物经过长时间的潜伏时期神经退化。这些结果符合其他研究认为朊病毒缺乏外国核酸。细胞的朊蛋白结构调查(PrPC)和朊蛋白痒病(PrPSc)表明可能构象的区别。 Conditions that diminished the beta-sheet content of PrPSc were the same as those identified previously that inactivate prion infectivity. Whether prion diversity as reflected by distinct "strains" producing different patterns of PrPSc accumulation is due to different conformers of PrPSc remains to be established. Advances in the purification and characterization of both PrPC and PrPSc seem to have identified the central event in PrPSc synthesis and prion propagation, ie, the unfolding of PrPC followed by its refolding into PrPSc. These findings underscore the fundamental features of prion structure and propagation that differentiate prions from other transmissible pathogens.