Book contents
- Frontmatter
- Contents
- Contributors
- 1 Introduction
- 2 Application of genetic epidemiology to dissecting host susceptibility/resistance to infection illustrated with the study of common mycobacterial infections
- 3 The diverse genetic basis of immunodeficiencies
- 4 Genetic diversity in the major histocompatibility complex and the immune response to infectious diseases
- 5 The cystic fibrosis transmembrane conductance regulator
- 6 The influence of inherited traits on malaria infection
- 7 Polymorphic chemokine receptor and ligand genes in HIV infection
- 8 NRAMP 1 and resistance to intracellular pathogens
- 9 The interleukin-12/interferon-γ loop is required for protective immunity to experimental and natural infections by Mycobacterium
- 10 Mannose-binding lectin deficiency and susceptibility to infectious disease
- 11 Blood group phenotypes and infectious diseases
- 12 Genetics of human susceptibility to infection and hepatic disease caused by schistosomes
- 13 Genetic susceptibility to prion diseases
- Index
- Plate section
13 - Genetic susceptibility to prion diseases
Published online by Cambridge University Press: 14 August 2009
- Frontmatter
- Contents
- Contributors
- 1 Introduction
- 2 Application of genetic epidemiology to dissecting host susceptibility/resistance to infection illustrated with the study of common mycobacterial infections
- 3 The diverse genetic basis of immunodeficiencies
- 4 Genetic diversity in the major histocompatibility complex and the immune response to infectious diseases
- 5 The cystic fibrosis transmembrane conductance regulator
- 6 The influence of inherited traits on malaria infection
- 7 Polymorphic chemokine receptor and ligand genes in HIV infection
- 8 NRAMP 1 and resistance to intracellular pathogens
- 9 The interleukin-12/interferon-γ loop is required for protective immunity to experimental and natural infections by Mycobacterium
- 10 Mannose-binding lectin deficiency and susceptibility to infectious disease
- 11 Blood group phenotypes and infectious diseases
- 12 Genetics of human susceptibility to infection and hepatic disease caused by schistosomes
- 13 Genetic susceptibility to prion diseases
- Index
- Plate section
Summary
WHAT ARE PRION DISEASES?
Prion diseases are otherwise known as transmissible spongiform encephalopathies (TSE), which are fatal neurodegenerative disorders that occur in many species of mammal, including humans. They include the cattle disease bovine spongiform encephalopathy (BSE) and the human form Creutzfeldt–Jakob disease (CJD). The oldest recognised TSE is scrapie in sheep and goats which has been recorded in these animals albeit under different names since the eighteenth century. Other species that have since been diagnosed with a specific form of TSE disease are included in the list shown in Table 13.1. The list has been divided into two groups, those that are host specific and those that have occurred as a consequence of the United Kingdom's highly publicised BSE outbreak of the 1980s–1990s.
The name TSE is derived from the fact that these diseases are transmissible from one animal to another and among species, and they are pathologically defined by the appearance of spongelike vacuolation in the gray matter of the brain.
Experimental animal transmission studies in primates and rodents have been performed using material from cattle diagnosed with BSE. This has indicated, to the acceptance of the majority of the scientific community, that the latest form of TSE in humans, variant Creutzfeldt–Jakob disease (vCJD), is attributable to infection by the bovine prions found in cases of cattle with BSE. The route of infection from contaminated bovine products is as yet undetermined but is most probably through dietary consumption.
- Type
- Chapter
- Information
- Susceptibility to Infectious DiseasesThe Importance of Host Genetics, pp. 361 - 392Publisher: Cambridge University PressPrint publication year: 2003