They are known to cause a rare inheritable disease associated with sleeplessness and ultimately death, fatal familial insomnia.
Chaperone proteins such as Hsp104 aid in the propagation of URE3 and PSI, which are examples of these in yeast. They are resistant to degradation by proteases and are produced by mutations in the gene encoding for the PrP protein, which results in them possessing beta-sheets instead of alpha helices. They are known to cause a rare inheritable disease associated with sleeplessness and ultimately death, fatal familial insomnia. Also causing Creutszfeld-Jacobs disease in humans, these are, for 10 points, what entities which earned Stanly Prusiner the 1997 Nobel Prize for his discovery of these infectious proteins linked with kuru and mad cow disease.
Chaperone proteins such as Hsp104 aid in the propagation of URE3 and PSI, which are examples of these in yeast.
{prions}