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Prions are proteins that can adopt two different forms, a normal form
and a misfolded form. This may not seem unusual, since many proteins
are flexible and adopt different shapes. However, prions have another
unusual characteristic: the misfolded form of the prion can force
normal prions to change into the misfolded shape. In this way, a few
misfolded prions can corrupt a whole population of normal prions,
converting them one-by-one into the misfolded shape. This can have
deadly consequences, as the levels of misfolded proteins build up.
For instance, misfolding of the PrP prion causes fatal neural
diseases in humans and other mammals. To make things worse, misfolded
prions are infectious, so a small dose of misfolded prions can infect
and corrupt an entire organism.
In
citing
the PDB please refer to: H.M. Berman, J. Westbrook, Z. Feng, G.
Gilliland, T.N. Bhat, H. Weissig, I.N. Shindyalov, P.E. Bourne:
The Protein Data Bank. Nucleic Acids Research, 28
pp. 235-242 (2000).
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State University of New Jersey and the
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Institute of Diabetes and Digestive and Kidney Diseases (NIDDK).
