Prion Diseases

Christopher J. Silva

doi:10.1128/9781555815486.ch24

Chapter 24 : Prion Diseases

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Affiliations: 1: Foodborne Contaminants Research Unit, Agricultural Research Service, Western Regional Research Center, USDA, Albany, CA 94710

Content Type: Monograph

Publication Year: 2009

Category: Clinical Microbiology

Book DOI: 10.1128/9781555815486

Chapter DOI: 10.1128/9781555815486.ch24

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Abstract:

Transmissible spongiform encephalopathies (TSEs), or prion diseases, are unlike any other malady. Prion diseases have long, largely asymptomatic incubation periods. Although the name would suggest all TSEs are acquired, in fact most prion diseases have no apparent cause and are called sporadic. TSEs are caused by a novel contagion once referred to as a slow virus and now called a prion. Scrapie is the oldest known TSE; it was first described in Europe at least 250 years ago. The presence of amyloid in the brains of kuru victims suggested a relationship with a much broader category of diseases. The relative resistance of a prion to proteinase K (PK) digestion is strain dependent. Worldwide, familial prion diseases account for 10 to 15% of all prion diseases. Kuru was eliminated when local authorities intervened to persuade members of the Fore tribe to stop ritual cannibalism. Diagnosing a prion disease is, by necessity, a diagnosis by elimination, since they are extremely rare. TSEs comprise a set of rare fatal neurological diseases found in many mammals and include such human diseases as Creutzfeldt-Jakob disease (CJD), Gerstmann-Straussler-Scheinker (GSS), fatal familial insomnia (FFI), and kuru.

Citation: Silva C. 2009. Prion Diseases, p 425-442. In Fratamico P, Smith J, Brogden K (ed), Sequelae and Long-Term Consequences of Infectious Diseases. ASM Press, Washington, DC. doi: 10.1128/9781555815486.ch24

Key Concept Ranking

Sodium Dodecyl Sulfate: 0.46095055
Enzyme-Linked Immunosorbent Assay: 0.46095055
Transmissible Spongiform Encephalopathies: 0.44020694
Magnetic Resonance Imaging: 0.438885
Bovine Spongiform Encephalopathy: 0.43624112
Chronic Wasting Disease: 0.43624112

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Prion Diseases

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/content/10.1128/9781555815486.ch24.ch24fig01

Figure 1.

Version of mouse PrPC (note that the sugar antennae are represented in their most complex form) ( 145 ).

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10.1128/9781555815486/ch24fig1.gif

Figure 1.

Version of mouse PrPC (note that the sugar antennae are represented in their most complex form) ( 145 ).

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Figure 2.

Catalog of polymorphisms found in human PrPC. Amino acid codes: A, alanine; D, aspartic acid; E, glutamic acid; F, phenylalanine; G, glycine; H, histidine; I, isoleucone), K, lysine; L, leucine; M, methionine; N, asparagine; P, proline; Q, glutamine; R, arginine; S, serine; T, threonine; V, valine; Y, tyrosine ( 114 , 145 ).

10.1128/9781555815486/ch24fig2_thmb.gif

10.1128/9781555815486/ch24fig2.gif

Figure 2.

References

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Tables

Prion Diseases

/content/10.1128/9781555815486.ch24.ch24tab01

Table 1.

WHO case definition for CJD ( 182 )

Table 1.

WHO case definition for CJD ( 182 )

/content/10.1128/9781555815486.ch24.ch24tab02

Table 2.

WHO case definition for vCJD ( 183 )

Table 2.

WHO case definition for vCJD ( 183 )

/content/10.1128/9781555815486.ch24.ch24tab03

Table 3.

WHO neuropathological criteria for CJD and other human TSE ( 181 , 183 )

Table 3.

WHO neuropathological criteria for CJD and other human TSE ( 181 , 183 )