Chaperones and prions

Published on September 4, 2008 Updated on September 26, 2020   42 min

Other Talks in the Therapeutic Area: Neurology

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Hello, my name is Yury Chernoff. I'm a professor at Georgia Institute of Technology, located in Atlanta, United States. Today, I'm going to talk about chaperones and prions.
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Prions were initially described as infectious agents which cause neurodegenerative diseases in humans and in other mammals. One example of such a disease is 'mad cow disease', which can be transmitted from cows to humans. Prion diseases are usually fatal and incurable diseases, therefore they represent a significant problem for the animal breeding industry and for human health.
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Perhaps the most interesting feature of prion diseases is an unusual mechanism of transmission. The prion infectious agent is composed of a protein in an abnormal shape. In the process of infection, the prion protein converts a non-prion host protein of the same amino acid sequence into a prion. Prion proteins form fiber-like aggregates enriched in beta-structures and cause amyloid. Recent models suggest that prion propagation represents a process of amyloid nucleated polymerization. In this way, prions resemble the other amyloid diseases and so-called 'neural inclusion disorders' such as Huntington's disease, Alzheimer's disease, and many others.