MHC class II signaling in antigen-presenting cells

Variant Creutzfeldt-Jakob disease (vCJD) was identified as a new disease in 1996. It was linked to infection with the bovine spongiform encephalopathy (BSE) agent although the epidemiological evidence for this was not strong, but later strain typing studies confirmed the association. The disease has affected predominantly young adults whose dietary and other characteristics are unexceptional compared to control groups, other than that all patients to date have been methoinine homozygous at codon 129 of the prion protein gene and the incidence has been about two times higher in the North of the UK. The number of cases in the 7 years after first identification of the disease has been considerably lower than initially feared, given the likely widespread exposure of the UK population to the BSE agent through contaminated beef products. Predictions of the possible future course of the epidemic have many associated uncertainties, but current mathematical models suggest that more than a few thousand cases is unlikely. Such modelling is limited by the absence of a test for infection with the vCJD agent. The development of a test that could be used on easily accessible tissue to detect infection early in the incubation period would not only advance understanding of the epidemiology of infection with the agent but would also aid the implementation of control measures to prevent potential iatrogenic spread.

The epidemiology of variant Creutzfeldt-Jakob disease.

Spiroplasma biofilm formation explains the role of these wall-less bacteria in the pathogenesis of transmissible spongiform encephalopathies (TSEs). Spiroplasma embedded in the biofilm polysaccharide matrix are markedly resistant to physical and chemical treatment, simulating the biologic properties of the TSE agent. Microcolonies of spiroplasma embedded in biofilm bound to clay are the likely mechanism of lateral transmission of scrapie in sheep and chronic wasting disease in deer via soil ingestion. Spiroplasma in biofilm bound to the stainless steel of surgical instruments may also cause iatrogenic transmission of Creutzfeldt-Jakob disease. Sessile spiroplasma in biofilm attach to the surface by curli-like fibrils, a functional amyloid that is important for spiroplasma entering cells. Curli fibers have been shown to interact with host proteins and initiate formation of a potentially toxic amyloid that multiplies by self-assembly. In TSE, this mechanism may explain how spiroplasma trigger the formation of prion amyloid. This possibility is supported by experiments that show spiroplasma produce α-synuclein in mammalian tissue cultures. The data linking spiroplasma to neurodegenerative diseases provide a rationale for developing diagnostic tests for TSE based on the presence of spiroplasma-specific proteins or nucleic acid. Research efforts should focus on this bacterium for development of therapeutic regimens for Creutzfeldt-Jakob disease.

/pdf/the-case-for-involvement-of-spiroplasma-in-the-pathogenesis-epnvi4jxtj.pdf

The case for involvement of spiroplasma in the pathogenesis of transmissible spongiform encephalopathies.

Prion diseases are fatal transmissible neurodegenerative disorders of both animals and humans associated with prolonged incubation periods and include scrapie, bovine spongiform encephalopathy (BSE) and Creutzfeldt-Jakob disease (CJD). The arrival of variant CJD (vCJD) and the recognition that it is causally related to BSE, to which there has been widespread dietary exposure, has lead to considerable public health concerns. According to the protein-only hypothesis, prions are principally or entirely composed of an abnormal isoform (PrP) of host-encoded cellular prion protein (PrP). Human prion diseases have inherited, sporadic and acquired aetiologies. The inherited prion diseases are all associated with coding mutations in the human PrP gene (PRNP) and PrP polymorphisms are known to affect susceptibility, incubation time and disease phenotype. Although PRNP is the major genetic determinant of prion disease susceptibility, it is becoming clear that other genes play an important role. Genetic studies in humans are limited by the small numbers of affected individuals and therefore to identify these genes several large mouse crosses have been analysed and multiple loci on at least eight different chromosomes now identified. To date, the regions identified are large and the identification of candidate genes remains challenging. However, the development of alternative mouse crosses offers the prospect of fine mapping, which, together with microarray analysis and increased sequence information, now makes identifying these susceptibility genes a realistic goal. Characterisation of these mouse alleles and then their human homologues may allow the identification of at-risk individuals for BSE prion infection, allow better prediction of any vCJD epidemic, and ultimately should identify new proteins and biochemical pathways which will contribute to our understanding of prion pathogenesis and provide new targets for therapeutic intervention.

Genetic Susceptibility to Prion Diseases in Humans and Mice

List of Contents.- Prion Protein and the Molecular Features of Transmissible Spongiform Encephalopathy Agents.- Past, Present and Future of Bovine Spongiform Encephalopathy in France.- Pathology and Pathogenesis of Bovine Spongiform Encephalopathy and Scrapie.- Public Health and the BSE Epidemic.- Clinical Features of Variant Creutzfeldt-Jakob Disease.- Neuropathology and Molecular Biology of Variant Creutzfeldt-Jakob Disease.- The Epidemiology of Variant Creutzfeldt-Jakob Disease.- Chronic Wasting Disease of Cervids.

Mad cow disease and related spongiform encephalopathies

HLA in French patients with variant Creutzfeldt-Jakob disease.

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HLA in French patients with variant Creutzfeldt-Jakob disease.