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.
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.
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.