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Chapter 25 : Bovine Spongiform Encephalopathy

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Abstract:

Bovine spongiform encephalopathy (BSE), widely known as "mad cow disease" is a subacute degenerative disease affecting the central nervous system (CNS) of cattle. This chapter talks about characteristics of the BSE agent, including typical and atypical BSE, stability of the infectious agent, and bodily distribution of infectivity. Much research has gone into defining the pathogenesis of BSE, the most important of which is studies in which cattle were infected orally and their tissues were examined at various times thereafter, up to and including the fully developed stage of illness. It is necessary to use caution in the interpretation of bioassay results from all nonbovine species, particularly if the results are based on ultrasensitive prion amplification techniques or bioassays in transgenic mice overexpressing the PrP gene. The chapter explains the characteristics of BSE in cattle (affected animals develop a progressive degeneration of the nervous system) and of variant form of Creutzfeldt-Jakob disease (vCJD) in humans (the young age at onset of illness with many adolescents afflicted). It is important that regulatory policies – protection of animal health and human health – be modified in accord with advances in experimental and epidemiologic knowledge to minimize adverse consequences to both animal and human health. In particular, the development of preclinical diagnostic tests may vastly improve the precision of proactive measures to minimize risks to animal and human health.

Citation: Brown P, Detwiler L. 2013. Bovine Spongiform Encephalopathy, p 651-671. In Doyle M, Buchanan R (ed), Food Microbiology. ASM Press, Washington, DC. doi: 10.1128/9781555818463.ch25
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Figure 25.1

Chronology of the TSEs. Bars represent known or presumed (striped) time periods of disease occurrence. Disease names are placed at time of first recognition (except for scrapie, first described in the 18th century). Dates within or under bars correspond to year of first experimental transmission in the laboratory; vertical arrows are placed at times of known or possible interspecies transmissions in nature. Horizontal arrows indicate probable extension of occurrence along time lines. CWD, chronic wasting disease; TME, transmissible mink encephalopathy; CJD, sporadic and familial forms of Creutzfeldt-Jakob disease; GSS, Gerstmann-Sträussler-Scheinker disease; FFI, fatal familial insomnia. doi:10.1128/9781555818463.ch25f1

Citation: Brown P, Detwiler L. 2013. Bovine Spongiform Encephalopathy, p 651-671. In Doyle M, Buchanan R (ed), Food Microbiology. ASM Press, Washington, DC. doi: 10.1128/9781555818463.ch25
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Image of Figure 25.2
Figure 25.2

Western blot patterns of PrP in sporadic (S) and variant (V) CJD brain tissue homogenates treated with proteinase K. MM, methionine homozygote; VV, valine homozygote. The vCJD pattern is distinct from both codon 129 genotypes of sCJD. Courtesy of Mark Head, CJD Surveillance Unit, Western General Hospital, Edinburgh, Scotland. doi:10.1128/9781555818463.ch25f2

Citation: Brown P, Detwiler L. 2013. Bovine Spongiform Encephalopathy, p 651-671. In Doyle M, Buchanan R (ed), Food Microbiology. ASM Press, Washington, DC. doi: 10.1128/9781555818463.ch25
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Image of Figure 25.3
Figure 25.3

Western blot patterns of PrP in typical classic (C) and in H and L types of atypical BSE brain tissue homogenates treated with proteinase K. In this blot the slight differences in the migration of the lowest band are more easily appreciated after treatment with -glycosidase (PNGase). Courtesy of Gianluigi Zanusso, Department of Neurosciences, University of Verona, Verona, Italy. doi:10.1128/9781555818463.ch25f3

Citation: Brown P, Detwiler L. 2013. Bovine Spongiform Encephalopathy, p 651-671. In Doyle M, Buchanan R (ed), Food Microbiology. ASM Press, Washington, DC. doi: 10.1128/9781555818463.ch25
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Image of Figure 25.4
Figure 25.4

The chronology of BSE in the United Kingdom (black) and EU (gray). Numbers of United Kingdom cases are 10× greater than scale. doi:10.1128/9781555818463.ch25f4

Citation: Brown P, Detwiler L. 2013. Bovine Spongiform Encephalopathy, p 651-671. In Doyle M, Buchanan R (ed), Food Microbiology. ASM Press, Washington, DC. doi: 10.1128/9781555818463.ch25
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Image of Figure 25.5
Figure 25.5

The chronology of BSE (black) and vCJD (gray) in the United Kingdom. Numbers of BSE cases are 1,000× greater than scale. doi:10.1128/9781555818463.ch25f5

Citation: Brown P, Detwiler L. 2013. Bovine Spongiform Encephalopathy, p 651-671. In Doyle M, Buchanan R (ed), Food Microbiology. ASM Press, Washington, DC. doi: 10.1128/9781555818463.ch25
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Figure 25.6

The chronology of vCJD in the United Kingdom (black) and EU (gray). doi:10.1128/9781555818463.ch25f6

Citation: Brown P, Detwiler L. 2013. Bovine Spongiform Encephalopathy, p 651-671. In Doyle M, Buchanan R (ed), Food Microbiology. ASM Press, Washington, DC. doi: 10.1128/9781555818463.ch25
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Image of Figure 25.7
Figure 25.7

Age at onset of cases of variant (black) and sporadic (gray) CJD in the United Kingdom, 1994 to 2005. Courtesy of Robert Will, CJD Surveillance Unit, Western General Hospital, Edinburgh, Scotland. doi:10.1128/9781555818463.ch25f7

Citation: Brown P, Detwiler L. 2013. Bovine Spongiform Encephalopathy, p 651-671. In Doyle M, Buchanan R (ed), Food Microbiology. ASM Press, Washington, DC. doi: 10.1128/9781555818463.ch25
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Image of Figure 25.8
Figure 25.8

Duration of illness of cases of variant (black) and sporadic (gray) CJD in the United Kingdom, 1994 to 2005. Courtesy of Robert Will, CJD Surveillance Unit, Western General Hospital, Edinburgh, Scotland. doi:10.1128/9781555818463.ch25f8

Citation: Brown P, Detwiler L. 2013. Bovine Spongiform Encephalopathy, p 651-671. In Doyle M, Buchanan R (ed), Food Microbiology. ASM Press, Washington, DC. doi: 10.1128/9781555818463.ch25
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Image of Figure 25.9
Figure 25.9

The pathognomonic vCJD “daisy plaque” consisting of a core of amyloid protein surrounded by vacuolar “petals.” Courtesy of James Ironside, CJD Surveillance Unit, Western General Hospital, Edinburgh, Scotland. doi:10.1128/9781555818463.ch25f9

Citation: Brown P, Detwiler L. 2013. Bovine Spongiform Encephalopathy, p 651-671. In Doyle M, Buchanan R (ed), Food Microbiology. ASM Press, Washington, DC. doi: 10.1128/9781555818463.ch25
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Figure 25.10

Red “RM” identification labels are used to differentiate between slaughterhouse tools used to handle SRMs and those used for edible products. Courtesy of Ana Carolina Alonso Simplicio de Oliveira, Frigoalta, Brazil. doi:10.1128/9781555818463.ch25f10

Citation: Brown P, Detwiler L. 2013. Bovine Spongiform Encephalopathy, p 651-671. In Doyle M, Buchanan R (ed), Food Microbiology. ASM Press, Washington, DC. doi: 10.1128/9781555818463.ch25
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Tables

Generic image for table
Table 25.1

Effectiveness of various chemical and physical inactivation treatments

Citation: Brown P, Detwiler L. 2013. Bovine Spongiform Encephalopathy, p 651-671. In Doyle M, Buchanan R (ed), Food Microbiology. ASM Press, Washington, DC. doi: 10.1128/9781555818463.ch25
Generic image for table
Table 25.2

Proteinase-resistant protein (PrP) and infectivity reductions under various pressure, temperature, and exposure time conditions

Citation: Brown P, Detwiler L. 2013. Bovine Spongiform Encephalopathy, p 651-671. In Doyle M, Buchanan R (ed), Food Microbiology. ASM Press, Washington, DC. doi: 10.1128/9781555818463.ch25
Generic image for table
Table 25.3

Misfolded-protein (PrP) reductions in various meat products containing 263K scrapie agent-infected hamster brain

Citation: Brown P, Detwiler L. 2013. Bovine Spongiform Encephalopathy, p 651-671. In Doyle M, Buchanan R (ed), Food Microbiology. ASM Press, Washington, DC. doi: 10.1128/9781555818463.ch25
Generic image for table
Table 25.4

Consumable bovine materials used by humans

Citation: Brown P, Detwiler L. 2013. Bovine Spongiform Encephalopathy, p 651-671. In Doyle M, Buchanan R (ed), Food Microbiology. ASM Press, Washington, DC. doi: 10.1128/9781555818463.ch25
Generic image for table
Table 25.5

Principal governmental measures taken to protect human and animal health

Citation: Brown P, Detwiler L. 2013. Bovine Spongiform Encephalopathy, p 651-671. In Doyle M, Buchanan R (ed), Food Microbiology. ASM Press, Washington, DC. doi: 10.1128/9781555818463.ch25

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