Infectious Human and Zoonotic Prion Diseases

Dear Colleagues,

Prion diseases are fatal transmissible spongiform encephalopathies affecting both animals and humans. While many animal species are susceptible to prion infection, animal prion diseases mainly include scrapie in sheep and goats, bovine spongiform encephalopathies (BSE, commonly named mad cow disease) in cattle, and chronic wasting disease (CWD) in elk and deer. Human prion diseases are very rare but highly heterogeneous. They can be sporadic, genetic, and acquired by infection, including Creutzfeldt–Jakob disease (CJD), kuru, Gerstmann–Sträussler–Scheinker disease, fatal insomnia, and variably proteinase-sensitive prionopathy. Whether there are any links in terms of the etiologies among the above various prion diseases remains unclear. However, it is known that they are all associated with the accumulation in the central nervous system of the infectious prion protein (PrP^Sc) that is derived from its cellular isoform (PrP^C) through a structural transition.

Due to the species barrier that is probably associated with differences in each host’s PrP sequences, prion diseases seem to be less transmissible across species. However, mad cow disease has been well documented to cause variant CJD (vCJD) in humans, the first proven zoonotic prion disease. The outbreak of mad cow disease and its zoonotic transmissibility raise concerns about the potential public health threat from other animal-derived prion diseases. For instance, one of the current main concerns is the uncertainty of CWD. It is the most contagious of all prion diseases, endemic in North America, and spreading to 26 US states and 3 Canadian provinces. The disease has also been reported in South Korea and recently in Europe. Its prevalence rates among free-ranging cervids are as high as 40% in some areas of the United States, where venison is mostly consumed. Furthermore, infectious prions are readily shed from infected cervids through urine, feces, saliva, and carcasses. Prions in these excreta and bodies have been found to remain stable and infectious in the environment for many years. Conceivably, CWD poses potential risks to public health in North America. There are several recent lines of evidence indicating that the zoonotic potential of CWD cannot be completely excluded. Given the huge damage of the COVID-19 pandemic and its unknown origin, infectious prions, their potential zoonotic nature, and possible outbreaks in the future cannot be underestimated.

This Special Issue welcomes prion researchers and experts to highlight the new developments in prions and their zoonotic potentials that have recently emerged. In addition, we also accept original research and review articles about the pathogenesis, diagnosis, and potential therapeutics of human and animal prion diseases. In particular, we seek manuscripts dealing with atypical prions and prion diseases that may be associated with any zoonotic potentials.

We look forward to your contributions and a valuable edition that will promote further understanding of this critical issue in the field.

Prof. Dr. Wen-Quan Zou
Dr. Zerui Wang
Guest Editors

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Pathogens is an international peer-reviewed open access monthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2700 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

• prions
• prion diseases
• prion-like
• zoonosis
• neurodegenerative disorders
• protein misfolding disorders
• protein aggregations
• biomarkers
• neuroscience
• Alzheimer’s disease
• Parkinson’s disease
• glycosylation