Prusiner was given a Nobel price for explaining prion biology, unique in our biosphere. Prions are almost impossible to destroy, following WHO, in order to attenuate them, you have to boil them in a 20% chlorine solution 3 atmospheres for 20mn. Prion is the agent for uncurable transmissible dementias in mammals, such as Variant Kreutzfeldt-Jakob in men. Since a whole business on human plasma had been set up in the USA, paying anonymous, untraceable donnors, with low control and almost no security checks, since this plasma is distributed world-wide by a Swiss company, I think it's time for meat industry to forget about their business fears and stop blocking research on prion, before we have to.
I did many conferences in Spain about Mad Cow Disease, which is also a prion disease. Researchers would come to me almost crying, explaining me how they were forced, all credits cut, to abandon their research on prion. I think meat industry is directly responsible.
Studying prions is difficult due to potential for contamination and disease spread. Even so, there are several funded and ongoing studies on prion diseases in the states. For specific ones I would search NIH reporter.
I would like to know more CWD prions and their propagation as an endemic northamerican disease. But I insist: studying prions is difficult due to meat industries that cut budgets off.
I think, due to our blood and plasma transfusion system, it is something we'd better study.
It is studied less because preventions have been put in place.
Rather strong claims have been made, like cooking does not destroy the adverse activity.
And even some prions showed to be still contaminant after burning them or cooking them. So, i doubt preventions are enough. They pasteurize blood plasma (60°) but it's clearly not enough against prions. Even not considering a possible animal contamination, such as CWD-hunters, stats shows "natural" Creutzfeldt-Jakob represent a major risk for blood transfusion. And many cases of quick Alzheimer or Parkinson in young people may be misdiagnosed.
First described TSE appeared in 1735 in United Kingdom. We still don't know why or how, but since then, prion diseases, on slow motion, didn't stopped to contaminate new species and new lands, taking advantage of human greed and newly born uncontrolled international trade. It is a very contaminant disease on a very large time scale. To compare with another neurodegenerative disease, rabbies are known and described since 5000 years and it affects only few species. Even after Mad Cow disease crisis, people and politicians and scientists managed to forget as if nothing ever happened.
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