Prions and prion diseases: Fundamentals and mechanistic details

Abstract

Prion diseases, often called transmissible spongiformneurological dysfunctions in many mamalian hosts. Priondiseases include Creutzfeldt-Jakob disease (CJD) in humans,bovine spongiform encephalopathy (BSE, “mad cow disease”)in cattle, scrapie in shep, and chronic wasting disease(CWD) in deer and elks. The cause of these fatal diseases is aproteinaceous pathogen termed prion that lacks functionalnucleic acids. As demonstrated in the BSE outbreak and itstransmission to humans, the onset of disease is not limited to ato another. Such a striking nature of prions has generated hugeconcerns in public health and attracted serious attention in thescientific comunities. To date, the potential transmission of prionsto humans via foodborne infection and iatrogenic routes hasnot been alleviated. Rather, the posible transmission ofhuman to human or cervids to human aggravates the terrifyingsituation acros the globe. In this review, basic features aboutprion diseases including clinical and pathological characteristics,recently accumulated evidences, the molecular and biochemicalaspects of prions, with an emphasis on the molecular interactionsinvolved in prion conversion that is critical during prionreplication and pathogenesis, are also addresed.