Prions: a threat to health security and the need for effective medical countermeasures

Abstract

Prions are infectious conformations of certain naturally occurring proteins. These misfolded proteins can structurally alter healthy protein, creating misfolded copies that repeat the process and form protein aggregates that lead to neuronal cell death. Although years can pass from initial prion infection to clinical presentation of symptoms, onset of symptoms is typically followed by rapid neurological decline resulting in death. Prion diseases have been characterized in animals ranging from sheep and cattle to cervids and humans, with notable cross-species infections such as the variant Creutzfeldt-Jakob disease. Thus, prions present a health risk with the potential to disrupt major food sources as well affect human health through animal to human and human to human transmission events. While human to human prion transmission is rare and the immediate risks for a prion-facilitated pandemic are low, prions are a class of pathogens for which we are underprepared. In addition, prions, and prion disease-like approaches, have also been discussed in the context of biological weapons and toxins, adding another layer of complexity surrounding biosecurity and biodefense. These threats underscore the need for increased scrutiny and research on prions. Here, pharmaceutical and nonpharmaceutical prion-specific interventions are discussed. Recent advances in prion therapeutic development are also briefly highlighted, and a set of policy recommendations are given that aims to provide high level suggestions for the prevention and mitigation of prion diseases.