Bovine spongiform encephalopathy BSE

AChR acetycholine receptor BSE bovine spongiform encephalopathy... 

Scientific procedures for risk assessment include assessment of risk for human health as well as risk for the environment. A substantial part of the EU risk assessment work was in 1997 delegated to the DG SANCO, in relation to the scandal surrounding BSE (bovine spongiform encephalopathy or mad cow disease ). Risk assessment work not under DG SANCO includes pharmaceuticals, working environment, and health effects caused by lifestyle factors such as diet, smoking, and alcohol consumption (EU 2006f). 

Following on from the BSE crisis, UKROFS included standards that related to this subject. These standards state In herds where animals have contracted BSE (bovine spongiform encephalopathy), or where animals have been brought in from herds in which BSE has occurred within the previous six years, then all contemporaries and first-generation progeny of all BSE cases must be removed from the herd and must not be sold as organic. Contemporaries are defined as animals originating from the same herd, which shared the same food, or were born into the same herd, or were bom within three months either side of the date of birth of the BSE case. 

In addition, the regulatory authorities are concerned about contamination with viruses and prions, such as the causative agent of bovine spongiform encephalopathy (BSE), which could be present in mammalian cell cultures. It is necessary to... 

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... 

Improving nutrition is essentially a process of encouraging people to make healthful choices that improve their well-being (Wansink, 2005). What happens, however, when we believe contamination, terrorism, or a genetic incidence threatens a part of the food supply Sometimes crises influence the recall, redesign, and communication efforts of individual companies (such as Tylenol, Perrier, Pilgrim s Pride). Others, such as the threat of mad cow disease (bovine spongiform encephalopathy, or BSE) in beef can compromise an entire industry. 

Fig. 3. Classification of human prion diseases. Sporadic the transformation from PrPc (circle) to PrPSc (square) occurs without apparent cause. Familial a point mutation ( ) is thought to facilitate the transformation. Infectious the transformation arises via PrPSc which acts as a template. The kinetic equations are defined by Eigen (1996). The infectious form includes kuru, iatrogenic CJD (iCJD), variant CJD (vCJD first reported in 1996), bovine spongiform encephalopathy (BSE first reported in 1985), and scrapie. In the nucleation-dependent model, monomeric PrPc and PrPSc are in chemical equilibrium.

The normal cellular form of prion protein (PrPc) can exist as a Cu-metalloprotein in vivo (492). This PrPc is a precursor of the pathogenic protease-resistant form PrPsc, which is thought to cause scrapie, bovine spongiform encephalopathy (BSE), and Creutzfeldt—Jakob disease. Two octa-repeats of PHGGGWGQ have been proposed as Cu(II) binding sites centered on histidine (493). They lack secondary and tertiary structure in the absence of Cu(II). Neurons may therefore have special mechanisms to regulate the distribution of copper. 

In certain cases, serum (fetal bovine serum—FBS) is added to promote the growth of cells. However, the bovine spongiform encephalopathy (BSE) problem has necessitated tight control on the quality of FBS (refer to Exhibit 10.12). This increases production and downstream processing costs. For new cell lines being developed, serum-free and protein-free media are used to circumvent the possibility of virus contamination from animal sources and the variation that may arise from use of serum from animal herds. 

Bovine spongiform encephalopathy (BSE or mad cow disease) is a progressive neurological degenerative disease in cattle. It is caused by a mutated protein called a prion. BSE was first reported in the United Kingdom in 1986. Creutzfeldt-Jakob disease (CJD) is a rare disease that occurs in humans. Evidence to date indicates it is possible for humans to acquire CJD after consuming BSE-contaminated cattle products. 

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,... 

Besides the direct and indirect risks associated with the consumption of agricultural products by humans in general, several risks are specific to the consumption of animal produce from modem agriculture. These have received considerable public attention in the past, i.e. antibiotic and hormone residuals, or just recently, such as Bovine Spongiform Encephalopathy (BSE). 

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