Transmissible spongiform encephalopathy

Transmissible Spongiform Encephalopathy (TSE ) ( German: " Transferable spongy brain suffering" ) is the name given to a series of brain disease ( encephalopathy ) in which there is a sponge-like changes in the brain tissue. Disorders can be found in both humans and animals. As polluter prions are accepted ( hence the name prion disease). The TSE are among the protein misfolding diseases.

TSE run always fatal; There is currently no treatment options available.

Forms of TSE

  • The oldest known TSE is scrapie ( scrapie ), an observed in sheep and goats spongy brain disease. It was discovered in 1732 in England.
  • Bovine spongiform encephalopathy ( BSE) in cattle, for the first time in late 1984 demonstrated in England
  • Chronic Wasting Disease (CWD ) in elk and other North American deer
  • Exotic ungulate encephalopathy (EUE ) in nyala and kudu Great (African antelope species )
  • Feline spongiform encephalopathy ( FSE): diagnosed in cats since 1990
  • Transmissible Mink Encephalopathy (TME ) in mink, the first in 1947 occurred on a mink farm in the U.S.

In addition, the following species may be affected by TSE: mules, elk, deer, oryx, chamois, rats, guinea pigs, hamsters, mice ( in laboratory tests ).

In humans, there are four TSE:

  • Creutzfeldt -Jakob disease (CJD)
  • Gerstmann - Sträussler - Scheinker syndrome ( GSSS )
  • Kuru
  • Fatal familial insomnia, the (English Fatal Familial Insomnia, FFI)

Etiology

Cause of the disease is most likely prions. For some TSE is known and proven that they are inherited. For the acquired form of prion transmission is assumed for the purposes of an infectious agent.

Pathology

In the brain of patients lacking any sign of an inflammatory or immune response. Characteristic features are filamentous, proteinaceous deposits in the nervous tissue and the spongy perforated structure of the brain.

Social significance

In the years 2000 to 2005 on TSE found on the front pages of the newspapers and in the main news on TV in Germany and other countries. In Europe, the awareness of TSE resulted in a medium-term change in the consumption of beef, and ( temporarily ) a significant decline in prices, in the course of which many companies had to change their production in the short term. The legislature of many countries and the European Union adopted rules, which should make animal products " TSE - safe" (eg BSE regulations in Germany ). These extend not only to meat products for food and animal feed, but also, for example gelatin capsule material in pharmaceuticals and even leather straps for orthopedic purposes of medical devices. In Germany, the prion research was initiated and promoted in 2001.

In South Korea, it came out in 2008 to nationwide mass scenes in connection with meat import relief from the United States, which also led to political consequences.

Proteopathy Kuru (disease)
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