Anatomy and Cell Biology 4429A Lecture Notes - Lecture 8: Oligomer, Neuroprotection, Scrapie

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Prion is not a form of a virus; rather it is a misfolded virus. It was first discovered in sheep and was called (cid:862) (cid:272)rapie(cid:863) si(cid:374)(cid:272)e the sheep (cid:449)ould s(cid:272)rat(cid:272)h the(cid:373)sel(cid:448)es o(cid:374) e(cid:448)er(cid:455) rough surfa(cid:272)e. it is k(cid:374)o(cid:449)(cid:374) to (cid:272)ause fatal neurodegenerative disorders. The protein in sheep that ga(cid:448)e rise to prio(cid:374)s, (cid:862)ju(cid:373)ped(cid:863) to (cid:272)o(cid:449)s. there is ~7 a(cid:373)i(cid:374)o a(cid:272)id differe(cid:374)(cid:272)e (cid:271)et(cid:449)ee(cid:374) (cid:272)o(cid:449)s a(cid:374)d sheep prio(cid:374), and the difference is much larger between humans and sheep. Prion strains refer to the different conformations adopted by prion proteins. Cooking and irradiation makes the proteins worse and there is currently no cure for the disease. The infectious form of the protein is denoted prpsc, and the normal form is denoted prpc. A harmless prion protein is converted to the infectious form due to abnormal tertiary folding these proteins have more than one stable conformations, and one of them is catalytic towards the normal conformation.

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