Prion diseases. An overview

Abstract

Prion disease is the new designation of a group of spongiform encephalopathies, all invariably fatal, which show similar clinical and neuropathological changes. They comprise a range of distinct diseases in both animals and man, and spontaneous, hereditary and transmissible forms are recognized. Until the sudden occurrence in the mid‐1980s of an epizootic of a formerly unknown disease, popularly named ‘mad cow disease’, in cattle in the UK, very little attention had been paid to these rather obscure diseases. Concurrently it was asserted that the disease‐causing agent appeared to be a ubiquitous mammalian brain constituent, and the disease mechanism a conformational change of its structure. These events have not only led to a new understanding of these extraordinary diseases, but have also provided insight into both neurodegeneration and disease mechanisms at the molecular level. Moreover, in 1997 the prion concept earned its originator the second Nobel price for medicine within this scientific field. In this introduction and overview of prion diseases, historical and philosophical perspectives are presented along with descriptions of the diseases in both animals and man. Epidemiology, genetics and transmissibility are also covered.

Exiting, enduring and innovating: Farm household adaptation to global zoonotic disease

“…First identified in England in 1986, BSE represents one of the most significant environmental disasters associated with the modern agro-food system (Leiss and Nicol, 2006). BSE is a fatal neurodegenerative prion disease (Dalsgaard, 2002) that is transmitted amongst cattle through the ingestion of BSE-infected central nervous system tissue. Although the recycling of animal materials as a high-protein feed source represents an effective way of reducing slaughterhouse waste to increase profits, the introduction of BSE-tainted animal materials into otherwise herbivorous bovine diets provided the BSE agent with a novel anthropogenic infection pathway (Smith and Bradley, 2003).…”

Characterization of the genomic region containing the Shadow of Prion Protein (SPRN) gene in sheep

“…These infectious particles are alternatively folded forms of the endogenous protein PrP C , encoded by PRNP [1,2]. Conversion of PrP C into PrP Sc requires the presence of PrP Sc and probably also of a not identified species-specific protein, 'protein X' [3,4].…”

Creutzfeldt-Jakob Disease with Paralysis of the Unilateral Vocal Cord and Soft Palate

“…In humans, the annual prevalence rate of prion disease is reported to be 1 case per 1,000,000 individuals (Dalsgaard 2002;Pedersen and Smith 2002). According to the cause, the disease is classified into 3 types: sporadic, familial, and infectious CJD.…”