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Telaah Lebih Jaun terhadap Prion: Protein Patologis sebagai Agen Penyakit
The term prion, proteinaceous infectious particle, introduced by Stanley B. Prusiner. Nobel prize winner in 1982 for infectious agents called prions that cause disease, which is also known as transmissible spongiform ancephalopathies (TSEs), is a group of (TSEs), is a group of diseases that are fatal neurogeneratif in mammals, arising either genetic, infectious, or sporadic. Called spongiform encephalopathies because the mammalian brain is effected by the disease looks like a sponge, due to accumulation of large vesicles. Prion diseases that have been known, both in animals (scrapie, mad cow diseases that have been known, both in animals (scraple, mad cow disease) and humans (Creutzfeldt -jacob Disease, Gerstman-straussler-Scheinker Syndrome, Kuru, and Fatal Insomnia), can be developed over the years and all have been fatal. Although 15 years ago, there was skepticism, when B. Prusiner first time revealed tat disease-causing agent of TSEs is a protein molecule called a prion. Nowadays more more can be prved that the prion protein (PrPSc), pathological forms of cellular prion protein (PrPC), many accumulate in the brains of people with TSE. Protein PrPC in its development may have misfolded (misfolding) that changed konformasinya forming PrPSc. This protein PrPSc deposits in the brain will eventually lead to the emergence of abnormalities in the nervous system.
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