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Generation and propagation of infectious prions by cyclic amplification of protein misfolding

Published on September 4, 2008 Archived on February 25, 2021   53 min

A selection of talks on Cell Biology

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0:00
Hello, my name is Claudio Soto, I'm Professor and Director of the Mitchell Center for Neurodegenerative Diseases at the Department of Neurology, University of Texas Medical Branch. I'll be talking today about the use of the cyclic amplification of protein misfolding for the generation and propagation of infectious prions.
0:19
One of the unique characteristic of prion diseases is that the prion protein can exist in two different stages. One is the normal prion protein that we call PrPc, 'c' stands for cellular. And this is a protein that we all have, all healthy people or animals have. The protein has a biological function, mainly alpha-helicoidal, sensitive to proteolysis and soluble. However, the same protein with the same sequence is also found in sick people or animals with a different folding. This is the so-called abnormal prion protein, and we call it PrPSc. This protein is supposed to be toxic and infectious, is rich in beta-sheet conformation, is resistant to proteolysis, and is insoluble.
1:05
The infectious agent associated to prion diseases, is supposed to be composed exclusively by the misfolded form of the prion protein, PrPSc, here in the figure represented in red squares. The way that the misfolded prion protein propagates a disease is by transforming the normal version of the protein PrPc, represented in the figure in green circles, gradually into its own. Gradually from the normal form into the misfolded form in a relatively slow process to get to the point in which sufficient amount of the normal protein has been transformed into the misfolded form, and this will produce tissue damage and disease. The period between the infection time to the time in which animals develop the disease is called incubation time, and this variable depends on the species. For example, in rodents this period can take several months, in cattle, for example, it can take several years, and in the case of humans it can take several decades.
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Generation and propagation of infectious prions by cyclic amplification of protein misfolding

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