Microbiology and Immunology 2500A/B Lecture Notes - Lecture 15: Chronic Wasting Disease, Transmissible Spongiform Encephalopathy, Neurodegeneration

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I(cid:374)fe(cid:272)tious age(cid:374)t that does(cid:374)"t ha(cid:448)e (cid:374)u(cid:272)lei(cid:272) a(cid:272)id (cid:894)(cid:373)ost i(cid:374)fe(cid:272)tious thi(cid:374)gs ha(cid:448)e (cid:374)u(cid:272)lei(cid:272) a(cid:272)ids(cid:895) Transmissible spongiform encephalopathies (tses) neurodegenerative diseases, always fatal, no available therapies. Transmissible: because they can be transmitted experimentally by inoculation. Spongiform: because microscopic vacuoles in brain tissue are a feature that help identify the disease. Encephalopathies: because these are diseases (-pathy) of the brain (encephalo-) Caused by infectious agents called (cid:862)p(cid:396)io(cid:374)s(cid:863: diagnosis of this is autopsy when due. The prion theory the (cid:862)p(cid:396)otei(cid:374) o(cid:374)l(cid:455)(cid:863) h(cid:455)pothesis fo(cid:396) (cid:272)(cid:396)apie (cid:449)as fi(cid:396)st p(cid:396)oposed (cid:271)(cid:455) ta(cid:374)le(cid:455) p(cid:396)uis(cid:374)e(cid:396) i(cid:374) (cid:1005)(cid:1013)(cid:1012)(cid:1006) this guy hypothesized that you can have a protein that causes i(cid:374)fe(cid:272)tious diseases (cid:894)does(cid:374)"t (cid:374)eed to ha(cid:448)e (cid:374)u(cid:272)lei(cid:272)(cid:895) Prions are a form of host protein found predominantly on the surface of neurons. The (cid:862)(cid:374)atu(cid:396)al(cid:863) p(cid:396)otei(cid:374) is (cid:272)alled p(cid:396)pc the cellular (c) prion protein. Prpc is a highly conserved protein in mammals the normal functions of prpc are not well understood - thought to be involved in synaptic function.

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