by Vincent Racaniello, PhD

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    Hello and welcome to Prions. After hearing this lecture, not only will you not want to eat meat any longer, but more importantly you should know the history of prion discovery. You should understand what prions are and what diseases they cause. You'll know that there are three different types of prion diseases and you'll begin to understand the threat of mad cow disease and chronic wasting disease to the food supply. The diseases caused by prions are called transmissible spongiform encephalopathies. Let's take that word apart, because it's quite long. An encephalopathy is a disease of the brain, and as you'll see in a moment, spongiform reflects the kind of changes caused in the brain and transmissible means that they can be infectious. So transmissible spongiform encephalopathies or TSEs; these are fatal neurodegenerative disorders of animals. They're uniformly fatal and they cause changes to your neurological system. Thousands of human are diagnosed with TSCs each year and about 1% of them arise by infection. We will talk about how the others are acquired as well. And as an example of a TSC, by 2002, 120 humans had contracted Creutzfeldt-Jacob disease from eating contaminated meat from animals with BSE, which stands for bovine spongiform encephalopathy. The TSC diseases affect many different animal species, we recognize TSEs of animals, so for example, bovine spongiform encephalopathy or BSC, mad cow disease, affects cows. Chronic wasting disease or CWD is a prion disease of deer, elk and moose. Exotic ungulate encephalopathy affects wild game like nyala and greater kudu, and there are also diseases of cats, feline spongiform encephalopathy. We also recognize human TSC diseases, for example Creutzfeldt-Jacob disease, fatal familial insomnia, Gerstmann-Straussler syndrome, Kuru and Variant CJD, all of these animal and human TSEs are caused by prions. Why are these so...

    About the Lecture

    The lecture Prions by Vincent Racaniello, PhD is from the course Microbiology: Introduction. It contains the following chapters:

    • Prions
    • Scrapie
    • Inactivation of Scrapie Infectivity
    • Current View of Prions
    • Sporadic CJD
    • Forced Cannibalism Spreads BSE
    • Chronic Wasting Disease

    Included Quiz Questions

    1. Kuru
    2. Chronic wasting disease
    3. Exotic ungulate encephalopathy
    4. Feline spongiform encephalopathy
    1. The pathogenic prion is a conformational isoform of a normal host protein
    2. These diseases occur only in humans
    3. Prions contain a small nucleic acid component
    4. Prions are highly sensitive to UV and ionizing radiation
    1. Consumption of contaminated beef is known to have transmitted a TSE to humans.
    2. Humans may sporadically develop prion disease
    3. Scrapie can be transmitted to mice
    4. Humans may transmit prion disease to other humans via corneal transplants
    1. They involve collections of several different parasites all working together.
    2. They are uniformly undetectable before symptoms develop.
    3. No diagnostic tests exist that can tell if a person is infected with a prion.
    4. There are no effective treatments to cure a prion disease.
    5. They involve an incubation period and symptoms which involve neurological problems.

    Author of lecture Prions

     Vincent Racaniello, PhD

    Vincent Racaniello, PhD

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