Human prion diseases and the prion protein - what is the current state of knowledge?

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.

Automated summary

The objective of this review article is to provide a comprehensive overview of the major features of human prion diseases, bridging the gap between basic research and clinical aspects. The article highlights current insights into the physiological functions of the prion protein and the process of pathological conversion. It discusses diagnostic, molecular, and clinical aspects of all human prion diseases, including rare diseases and potential transmission of prions from other species.