朊病毒病是致命的传染性神经退行性疾病和原型构象疾病，由正常细胞朊病毒蛋白 (PrP C ) 构象转变为病理性 PrP Sc同种型引起。例如绵羊和山羊的痒病、牛的牛海绵状脑病 (BSE)、鹿科动物的慢性消耗性疾病 (CWD) 以及人类的克雅氏病 (CJD)。目前尚无可用的治疗方法，而 BSE 和 CWD 等动物朊病毒疾病会对经济、生态、动物健康甚至人类健康产生负面影响。 BSE 已被证实对人类健康构成威胁，越来越多的证据支持 CWD 具有人畜共患病的可能性。 CWD 在北美的数量和分布不断扩大，最近在斯堪的纳维亚国家被发现。 CWD 是唯一一种同时发生在野生和养殖动物身上的朊病毒疾病，加上传染性广泛传播到环境中，阻碍了遏制策略。目前正在大力推动开发针对 CWD 的疫苗，包括可用于野生动物的疫苗。免疫系统不会针对朊病毒感染产生真正的免疫反应，因为 PrP C和 PrP Sc具有相同的蛋白质一级结构，而朊病毒似乎并不代表免疫反应的触发因素。这就需要替代的疫苗策略，其重点是 PrP C导向的自身抗体或疾病特异性结构和表位的暴露。几个小组已经建立了概念验证，即此类候选疫苗可以在鹿科动物和啮齿动物模型中诱导一定水平的保护性免疫，而不会引起不必要的副作用。 本次审查将重点介绍最新进展并讨论剩余的进展和挑战。
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Vaccines for prion diseases: a realistic goal?
Prion diseases are fatal infectious neurodegenerative disorders and prototypic conformational diseases, caused by the conformational conversion of the normal cellular prion protein (PrPC) into the pathological PrPSc isoform. Examples are scrapie in sheep and goat, bovine spongiform encephalopathy (BSE) in cattle, chronic wasting disease (CWD) in cervids, and Creutzfeldt–Jacob disease (CJD) in humans. There are no therapies available, and animal prion diseases like BSE and CWD can negatively affect the economy, ecology, animal health, and possibly human health. BSE is a confirmed threat to human health, and mounting evidence supports the zoonotic potential of CWD. CWD is continuously expanding in North America in numbers and distribution and was recently identified in Scandinavian countries. CWD is the only prion disease occurring both in wild and farmed animals, which, together with extensive shedding of infectivity into the environment, impedes containment strategies. There is currently a strong push to develop vaccines against CWD, including ones that can be used in wildlife. The immune system does not develop a bona fide immune response against prion infection, as PrPC and PrPSc share an identical protein primary structure, and prions seem not to represent a trigger for immune responses. This asks for alternative vaccine strategies, which focus on PrPC-directed self-antibodies or exposure of disease-specific structures and epitopes. Several groups have established a proof-of-concept that such vaccine candidates can induce some levels of protective immunity in cervid and rodent models without inducing unwanted side effects. This review will highlight the most recent developments and discuss progress and challenges remaining.