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Classical bovine spongiform encephalopathy (BSE) is an acquired prion disease that is invariably fatal in cattle and has been implicated as a significant human health risk. Polymorphisms that alter the prion protein of sheep or humans have been associated with transmissible spongiform encephalopathy susceptibility or resistance. In contrast, there is no strong evidence that non-synonymous mutations in the bovine prion gene (PRNP) are associated with classical BSE disease susceptibility. However, two bovine PRNP insertion/deletion polymorphisms, one within the promoter region and the other in intron 1, have been associated with susceptibility to classical BSE. These associations do not explain the full extent of BSE susceptibility, and loci outside of PRNP appear to be associated with disease incidence in some cattle populations. To test for associations with BSE susceptibility, we conducted a genome wide scan using a panel of 3,072 single nucleotide polymorphism markers on 814 animals representing cases and control Holstein cattle from the United Kingdom BSE epidemic. A higher resolution, 50K SNP, genome-wide scan was used with a sub-set (N=330) of animals to test for association with BSE incidence. Further, the bovine prion gene was tested, 19 haplotype tagging SNPs and 2 insertion/deletion alleles for association with BSE disease incidence. The Holstein case and control cattle analyzed here consisted of two sets, one set with known family relationships and the second set with BSE cases with paired controls. The family set comprised of half-sibling progeny from six sires, revealed 27 SNPs representing 18 chromosomes associated with incidence of BSE disease, confirming previously reported chromosomal regions. Further analysis of the BSE case and paired control samples using the 50K SNP chip a polymorphic locus was identified on chromosome 1 at 29,147,078 bp with a moderate significant association (p=3.09E-5) and over-represented in BSE affected animals. Further, analysis of the prion gene and the paired BSE case and control animals revealed a haplotype within the region of high LD to associate with BSE un-affected animals (p-value =0.000114). It is clear from our analyses that several regions of the genome are statistically associated with the incidence of classical BSE in European Holstein cattle. / Animal Science

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No description available.

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The emergence of Bovine Spongiform Encephalopathy (BSE) or “mad cow” disease has forced new practices in raising of cattle, risk management in abattoirs, marketable cuts of meat and disposal of potentially infective material. The Canadian Food Inspection Agency defines BSE as a progressive, fatal disease of the nervous system of cattle. In 1996 BSE became a human health issue when a link was discovered between BSE and a new variation of Creutzfeldt-Jacob disease (vCJD), a devastating and incurable disease with a very low-probability of infection but a high fatality rate (Collinge, 1999). To avert further BSE and potential vCJD cases, new policies need to be implemented (CFIA, 2007b; CFIA, 2007b; CFIA, 2008; DEFRA, 2004; OIE, 2007). These policies would not only protects the health of consumers in Canada, they are a prerequisite to exporting Canadian meat products. Failure to enact risk reduction measures has had devastating economic impacts (FDA, 2005; Hill, 2005; Mitura & Di Pietro, 2004; Poulin & Boame, 2003). However, not all technologies being used to manage the risk of prion diseases are deemed effective, and many have strong economies of scale which if implemented may well exclude small scale farming and slaughterhouses, unless consumers accept much higher cost products. Creating an effective management plan for animal by-products (ABPs) is a complex issue involving multiple conflicting objectives. In order to meet the objectives, the CFIA has approved five management options that offer varying levels of risk management while imposing different environmental, social and economic costs. The costs of these are linked to the operational scale and technology being considered. Furthermore, stakeholders are likely to be sensitive to different attributes of these options and design of successful policies. The focus of this research is on the tradeoff between managing the human health risk of exposure to the BSE prion and the economics of managing this risk while retaining consumer demand. The challenge lies in discovering alternative means of managing livestock waste tissue that are practical for producers and regulators and are attractive to consumers. This challenge was addressed by asking the following two questions: 1. What is the cost and effectiveness of different waste disposal options for British Columbia? 2. What is the extent of consumer willingness to share in the costs of increased food safety? In answering these questions a two stage methodology was designed. The first stage was a technological analysis whereby each was characterized and compared to the extent in which they satisfied operational objectives. The second stage was conducted through an online survey whereby we gather information on the following three broad categories, demographics, determinants of purchasing behaviour and willingness to pay for varying levels of food safety. The results of the technological analysis show that the technology of choice varies based on stakeholder preference. The survey results confirm earlier results that consumers value food safety and they are willing to pay to mitigate food safety risks (Hammitt, 1990; Latouche, Rainelli, & Vermersch, 1998; Loureiro, McCluskey, & Mittelhammer, 2003; McCluskey, Grimsrud, Ouchi, & Wahl, 2005; Röhr, Lüddecke, Drusch, Müller, & Alvensleben, 2005) Within the context of beef selection survey respondents are willing to pay up close to 184 cents per pound of beef more than they are currently paying and the study has highlighted the following two predictors of for this tendency: 1. Consumer willingness to pay for organic food and: 2. Respondent level of concern regarding food borne illnesses In terms of policy selection, regulations in BC should impose risk reduction measures that achieve considerable levels of risk management, communicate this clearly to the public as well as the impact of these measures on production costs and provide a means whereby consumers can select for this attribute, such as a labeling program.

Transmissible spongiform encephalopathies (TSEs), or prion diseases, are a group of inevitably fatal neurodegenerative diseases that occur in mammalian species. Ovine susceptibility to scrapie, the prototypical TSE, is predominantly modulated by nonsynonymous polymorphisms within exon 3 of the ovine prion protein gene (PRNP). Investigation of PRNP exon 3 for two hair-sheep breeds revealed a novel predicted amino acid substitution (P116) associated with the ovine ARQ allele (P116A136R154Q171). Additionally, two novel ovine PRNP genotypes (PARQ/ARR; PARQ/ARQ) also were detected, and most of the hair sheep sampled possessed PRNP exon 3 genotypes associated with some degree of resistance to scrapie and/or experimental BSE (bovine spongiform encephalopathy). Unlike sheep, expression of bovine spongiform encephalopathy (BSE) in cattle and other bovids has not been associated with nucleotide variation within bovine PRNP exon 3. However, BSE susceptibility has been tentatively associated with specific insertion-deletion (indel) polymorphisms within the putative bovine PRNP promoter, and to a lesser extent intron 1, for a few German cattle breeds. Evaluation of the patterns of nucleotide variation associated with bovine PRNP exon 3 provided evidence that strong purifying selection has intensely constrained bovine exon 3 over the long-term evolutionary history of the subfamily Bovinae, as well as evidence for significant purifying selection in regions of bovine PRNP exon 3 that are considered to be of functional, structural, and pathogenic importance in other mammalian species. Evaluation of the frequencies of known indel polymorphisms within the putative bovine PRNP promoter for a panel of U. S. cattle sires revealed no significant differences in the distribution of promoter alleles and/or genotypes between U. S. cattle sires and BSEaffected German cattle. Notably, a nonsynonymous PRNP exon 3 polymorphism (T50C) identified in American bison (Bison bison) was tentatively associated with Brucella spp. seropositivity. Specifically, a significant overabundance (P = 0.021) of Yellowstone National Park bison possessing the CC genotype were Brucella spp. seropositive. Furthermore, the T-allele and TT genotype were observed at significantly higher frequencies in three bison populations that were either founded from Brucella spp. seronegative stock or previously subjected to test-and-slaughter management to eradicate brucellosis.

The emergence of Bovine Spongiform Encephalopathy (BSE) or “mad cow” disease has forced new practices in raising of cattle, risk management in abattoirs, marketable cuts of meat and disposal of potentially infective material. The Canadian Food Inspection Agency defines BSE as a progressive, fatal disease of the nervous system of cattle. In 1996 BSE became a human health issue when a link was discovered between BSE and a new variation of Creutzfeldt-Jacob disease (vCJD), a devastating and incurable disease with a very low-probability of infection but a high fatality rate (Collinge, 1999). To avert further BSE and potential vCJD cases, new policies need to be implemented (CFIA, 2007b; CFIA, 2007b; CFIA, 2008; DEFRA, 2004; OIE, 2007). These policies would not only protects the health of consumers in Canada, they are a prerequisite to exporting Canadian meat products. Failure to enact risk reduction measures has had devastating economic impacts (FDA, 2005; Hill, 2005; Mitura & Di Pietro, 2004; Poulin & Boame, 2003). However, not all technologies being used to manage the risk of prion diseases are deemed effective, and many have strong economies of scale which if implemented may well exclude small scale farming and slaughterhouses, unless consumers accept much higher cost products. Creating an effective management plan for animal by-products (ABPs) is a complex issue involving multiple conflicting objectives. In order to meet the objectives, the CFIA has approved five management options that offer varying levels of risk management while imposing different environmental, social and economic costs. The costs of these are linked to the operational scale and technology being considered. Furthermore, stakeholders are likely to be sensitive to different attributes of these options and design of successful policies. The focus of this research is on the tradeoff between managing the human health risk of exposure to the BSE prion and the economics of managing this risk while retaining consumer demand. The challenge lies in discovering alternative means of managing livestock waste tissue that are practical for producers and regulators and are attractive to consumers. This challenge was addressed by asking the following two questions: 1. What is the cost and effectiveness of different waste disposal options for British Columbia? 2. What is the extent of consumer willingness to share in the costs of increased food safety? In answering these questions a two stage methodology was designed. The first stage was a technological analysis whereby each was characterized and compared to the extent in which they satisfied operational objectives. The second stage was conducted through an online survey whereby we gather information on the following three broad categories, demographics, determinants of purchasing behaviour and willingness to pay for varying levels of food safety. The results of the technological analysis show that the technology of choice varies based on stakeholder preference. The survey results confirm earlier results that consumers value food safety and they are willing to pay to mitigate food safety risks (Hammitt, 1990; Latouche, Rainelli, & Vermersch, 1998; Loureiro, McCluskey, & Mittelhammer, 2003; McCluskey, Grimsrud, Ouchi, & Wahl, 2005; Röhr, Lüddecke, Drusch, Müller, & Alvensleben, 2005) Within the context of beef selection survey respondents are willing to pay up close to 184 cents per pound of beef more than they are currently paying and the study has highlighted the following two predictors of for this tendency: 1. Consumer willingness to pay for organic food and: 2. Respondent level of concern regarding food borne illnesses In terms of policy selection, regulations in BC should impose risk reduction measures that achieve considerable levels of risk management, communicate this clearly to the public as well as the impact of these measures on production costs and provide a means whereby consumers can select for this attribute, such as a labeling program.

Zugl.: Kiel, Univ., Diss., 2005