Transmissible spongiform encephalopathies

@article{Collins2004TransmissibleSE,
  title={Transmissible spongiform encephalopathies},
  author={Steven John Collins and Victoria A. Lawson and Colin L. Masters},
  journal={The Lancet},
  year={2004},
  volume={363},
  pages={51-61}
}
Nosologically, transmissible spongiform encephalopathies (TSE or prion diseases) should be grouped with other neurodegenerative disorders such as Alzheimer's and Parkinson's diseases, which are all caused by toxic gain of function of an aberrant form of a constitutively expressed protein. Failure to clear these proteins from the brain induces neuronal dysfunction. Transmissibility is the property that separates TSE from other neurodegenerative diseases, and this property seems to reside within… Expand
Transmissible spongiform encephalopathies
TLDR
Treatment of TSE is purely symptomatic and there is no presymptomatic or early test for diagnosis, but periodic electroencephalography or 14-3-3 protein detection in spinal fluid are helpful for clinical diagnosis. Expand
Transimissible Spongiform Encephalopathies
TLDR
The most widely accepted hypothesis on the nature of the infectious agent causing transmissible spongiform encephalopathies (TSEs) predicates that it consists of a scrapie-like prionprotein (PrPSc), an abnormally folded, protease-resistant, beta-sheet-rich isoform of a normal cellular prion protein (PrPC). Expand
42 – Prion Diseases
TLDR
Creutzfeldt– Jakob disease (CJD) is today's most prevalent human prion disease, which is exceedingly rare, and several scenarios may account for the increase in incidence, including improved reporting, iatrogenic transmission, and transmission of a prion zoonosis. Expand
Species barriers in prion diseases--brief review.
TLDR
Studies done with transgenic animals, tissue culture, and cell-free assays established PrP as being necessary for TSE pathogenesis and illustrated that certain amino acid residues are more influential than others for conferring resistance to TSE agent transmission. Expand
Introduction to the transmissible spongiform encephalopathies or prion diseases.
  • B. Chesebro
  • Biology, Medicine
  • British medical bulletin
  • 2003
TLDR
Understanding of the structure of the disease-associated protease-resistant PrP should help elucidate the mechanism of PrP conversion from the normal to the abnormal form and open up new approaches to both diagnosis and therapy. Expand
[Human prion diseases: current issues].
TLDR
Because of the lack of effective treatment, prevention of both alimentary prion infections (consumption of contaminated meat products) and transmissible iatrogenic infections (the use of biopreparations from animal tissues) is important. Expand
CHAPTER 22 – Transmissible Spongiform Encephalopathies
TLDR
The primary focus of this chapter is on bovine spongiform encephalopathy and its assumed relationship with vCJD and more detailed summaries on scrapie and chronic wasting disease (CWD). Expand
Prions and neuro degenerative diseases
TLDR
Currently, definite diagnosis of prion diseases is still considered to be possible only after histopathological examination of biopsied or autopsied brain material, and no treatment is available, although research in this area is progressing. Expand
Prion Diseases
TLDR
The geographical distribution and the temporal trends of CJD and vCJD; the major events in the pathogenesis of prion diseases; the risk factors for sporadic CJD; and the possible strategies for treating them are reviewed. Expand
A SHORT REVIEW OF TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES, AND GUIDELINES FOR MANAGING RISKS ASSOCIATED WITH CHRONIC WASTING DISEASE IN CAPTIVE CERVIDS IN ZOOS
  • D. Travis, Michele A Miller
  • Biology, Medicine
  • Journal of zoo and wildlife medicine : official publication of the American Association of Zoo Veterinarians
  • 2003
TLDR
A short review of the transmissible spongiform encephalopathies of immediate concern to zoo and wildlife veterinarians and wildlife biologists and suggests risk management strategies for the prevention of these diseases, with special focus on chronic wasting disease of cervids in North America. Expand
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