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Kits for Prionen Proteins


n past twenty years public awareness of prion diseases has grown in parallel with the occurrence of cases of BSE and realization that this disease can be transmitted to humans through the consumption of contaminated meat and give rise to a variant form of Creutzfeldt-Jakob disease (CJD) the prion disease in humans - rarer forms of manifestation are known as Gerstmann-Sträussler-Schenker Syndrome (GSS) and Fatal Familial Insomnia (FFI). Better known prion diseases in livestock are “Scrapie” in sheep and goats, Bovine Spongiform Encephalopathy (BSE) in cattle and Chronic Wasting Disease (CWD) in elk. Prion diseases are characterized by the aggregation of PrPsc within the central nervous system. These aggregates can be visualized and are often referred to “plaques”. The toxic accumulation of PrPsc in turn causes the formation of large intracellular holes (vacuole formation) and neuronal death. However, once symptoms appear the disease progresses rapidly, leading to widespread brain damage. Neurodegenerative symptoms can include dementia, ataxia (dysfunction of balance and coordination), convulsions and behavioural or personality changes. Death typically occurs within one to three years from the onset of clinical symptoms. Moreover prions are in focus of science regarding their function for other neurodegenerative or cognitive diseases.