For an explanation, see the main splats page
SPLATS about prionic diseases
The principles of prionic diseases
- Proteins are made of folded chains of amino acids: prions are misfolded proteins, causing a variety of 'prionic' diseases which attack the brain.
- In technical language, prion diseases are a family of rare progressive neurodegenerative disorders affecting both humans and animals.
- That means the prion diseases begin slowly and get worse over time, attacking the nervous system of the animal they attack.
- Known prionic diseases include kuru, Creutzfeldt-Jakob disease (and vCJD) in humans, scrapie in sheep, CWD in deer and BSE or 'mad cow disease' in cattle.
- Scrapie was the first disease known, and for a very long time, scientists believed that the disease could not cross what they called 'the species barrier'.
- Scientists now believe that BSE was caused when ground-up nerve tissue from diseased sheep was fed to cattle as a food supplement.
- Scientists strongly suspect that vCJD (variant Creutzfeldt-Jakob disease) in humans was caused by eating cows suffering from BSE.
- Other human prion diseases are: GSS (Gerstmann-Straussler-Scheinker syndrome); FFI (Fatal familial Insomnia) and Alpers Syndrome.
- Prions can pass on their misfolding to other proteins, causing prionic diseases to spread. The only answer is to destroy all carcases of diseased animals.
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