Prion diseases and the prion protein are only partially understood so far in many aspects. This explains the continued research on this topic, calling for an overview on the current state of knowledge. The main objective of the present review article is to provide a comprehensive up-to-date presentation of all major features of human prion diseases bridging the gap between basic research and clinical aspects. Starting with the prion protein, current insights concerning its physiological functions and the process of pathological conversion will be highlighted. Diagnostic, molecular, and clinical aspects of all human prion diseases will be discussed, including information concerning rare diseases like prion-associated amyloidoses and Huntington disease-like 1, as well as the question about a potential human threat due to the transmission of prions from prion diseases of other species such as chronic wasting disease. Finally, recent attempts to develop future therapeutic strategies will be addressed.

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人类朊病毒疾病和朊病毒蛋白——目前的知识状况如何？

迄今为止，朊病毒疾病和朊病毒蛋白在许多方面仅得到部分了解。这解释了对该主题的持续研究，要求对当前知识状况进行概述。本综述文章的主要目的是提供人类朊病毒疾病所有主要特征的全面最新介绍，以弥合基础研究和临床方面之间的差距。从朊病毒蛋白开始，将重点介绍目前对其生理功能和病理转化过程的认识。将讨论所有人类朊病毒疾病的诊断、分子和临床方面，包括有关朊病毒相关淀粉样变性和亨廷顿病样1等罕见疾病的信息，以及由于朊病毒传播而对人类造成潜在威胁的问题。其他物种的朊病毒疾病，例如慢性消耗性疾病。最后，将讨论最近开发未来治疗策略的尝试。