《生命科学》 2019, 31(11): 1133-1139
摘 要：摘 要：传染性海绵状脑病(transmissible spongiform encephalopathy) 或朊病毒疾病是一类致死的神经退行性疾病，由朊病毒蛋白(prion protein) 的错误折叠而引起，影响包括人在内的多种哺乳动物。朊病毒蛋白由宿主基因PRNP 编码，正确折叠的蛋白质不仅没有致病性和感染性，还具有重要的生理功能；其错误折叠后则会转变为具有感染性的朊病毒(prion)，能够使许多哺乳动物患病。但近年来，也有不少研究报道兔、犬、马可以抵御朊病毒疾病。由于种群内自然病例的缺乏和朊病毒感染动物实验的失败，这三种哺乳动物被公认为具有朊病毒抗性。现简要介绍朊病毒疾病的概念和分类，对抵御朊病毒感染的兔、犬、马可能的抗病机制进行综述，并讨论与展望当前对朊病毒疾病有特殊抗性物种的研究中存在的机遇与挑战。
Abstract: Abstract: Transmissible spongiform encephalopathy or prion disease， caused by misfolding of the prion protein， is a kind of fatal neurodegenerative disease that affects many mammals including humans. Prion protein is encoded by the host gene PRNP， and the correctly folded protein not only does not have pathogenic and infectious functions but also has important physiological functions. However， it can be transformed into prion after misfolding. Prion is uniquely infectious and many mammals are susceptible to its infection. Some studies have recently reported that a few mammals (rabbits， dogs and horses) exhibit resistance to prion disease. These three mammal species are recognized as prion-resistant mammals as no successful natural or experimental challenge infections have been reported. This article briefly introduces the concept and classification of prion disease， and provides a review on possible mechanisms proposed for explaining why rabbits， dogs， and horses are resistant to prion infection. Finally， we discuss and prospect opportunities and challenges in current study of mammal species exhibiting resistance to prion disease.
