Bovine related prion diseases

The prion diseases are a closely related group of neuro-degenerative conditions which affect both humans and animals. They have previously been described as the subacute spongiform encephalopathies, slow virus diseases and transmissible dementias, and include scrapie in sheep, bovine spongiform encephalopathy (BSE) in cattle, and the human prion diseases, Creutzfeldt-Jakob disease (CJD), Gerstmann-Straussler-Scheinker disease (GSS), fatal familial insomnia (FFI) and kuru. Prion diseases are... 

With the background of the mad cow crisis in Europe, questions relating to the prion diseases have attracted intensive interest. It is now widely accepted that prion diseases, such as Creutzfeldt-Jakob disease (CJd) in humans and bovine spongiform encephalopathy (BSE) are caused by a conformational change of the prion protein (PrP) from a normally folded cellular form, PrP ", to an alternate, aggregation-prone, pathogenic scrapie form,... 

Human blood, given by blood donors, is a source not only of cellular material (red cells, white cells, platelets) but also of blood plasma, which is subjected to protein fractionation to give albumin, anti-hemophilia factors and immunoglobulins. The possibility of transmission of Creutzfeld-Jakob disease (CJD) - prions are not really understood - and acquired immune deficiency syndrome (AIDS), if sterilization procedures fail, combined with the feasibility of manufacturing proteins by recombinant DNA technology, mean that there is uncertainty about the long-term future of the blood products industry. Indeed, the problems with bovine spongiform encephalitis (BSE) and the related fatal human brain disorder, CJD, have led to unease about cattle-derived substances. 

Aggregation of protein molecules may be held responsible for the malfunctioning of cellular processes. For instance, conformational diseases such as bovine spongiform encephalopathy (BSE), Creutzfeldt-Jakob s, Parkinson s, and Alzheimer s seem to be related to the aggregation of prion-like or amyloid proteins. 

Another group of organisms that straddle the life - non-life border are the prions. These have come to public attention recently because of their controversial association with Creutzfeld-Jacob disease (CJD), a fatal infection of the brain in humans, so-called scrapie in sheep, and bovine spongiform encephalopathy (BSE), a related disease in cattle, as discussed by Heaphy (2004) and in Prusiner (2004). Several other neurological diseases in animals and humans are also linked with prions which are proteinaceous particles and thus earbon-based. Unlike other disease-causing organisms, prions do not contain nucleic acids and consist of modified forms of normal cellular protein. They ean be ineubated in brain tissue for long periods before they have an effeet whieh involves impairment of tissue function and ultimately impairment of human/animal funetion prior to death. How prions enter the body and infeet brain tissue has not yet been aseertained in detail. 

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