Prions. Although that word may sound foreign to many readers, the words, “mad cow disease” may sound less so. From 1986 to 2001, this disease spread throughout the United Kingdom and even to Canada, turning cows into twitchy, aggressive animals with wildly varying weights and gaits. As you may have guessed, prions are the cause of mad cow disease, or “bovine spongiform encephalopathy (BSE),” as it is more formally known.

Prions are a group of infectious agents that are unique for their route of infection. Prions are not viruses, bacteria, or fungi, but are misfolded proteins. They are usually harmless when in their normal forms in animals. However, extremely rare cases of prion protein mutation within an organism may cause diseases known as transmissible spongiform encephalopathies (TSEs), such as Creutzfeldt-Jakob Disease in humans, BSE in cows, and scrapie in sheep. TSEs can spread in rare cases between organisms. For example, if a person ingests a steak contaminated with BSE, the person may develop what is known as variant-CJD.

With long incubation periods lasting up to a decade, devastating effects, and relative lack of research, the word “prion” has typically been associated with an obscure but deadly and incurable disease.

However, a recent discovery by researchers at Stanford University School of Medicine may shed new light on this mysterious class of protein. Those researchers found that prion proteins in yeast, and comparable proteins in humans, may help their respective organisms survive in the world. According to the study, yeast cells may use their prions when under duress. For example, during elevated heat conditions, the yeast cells can generate proteins known as molecular chaperones that fold prions within the yeast cell into a desired shape. From there, other prion proteins can also quickly adapt accordingly in a cascading reaction.

In general, the function of a protein directly relates to its shape and vice versa: it’s how a single mutated protein can ravage the nervous system of an infected animal. In the case of yeast cells, however, researchers found that newly folded yeast proteins may confer beneficial traits such as improved growth or a resistance to heat stress to the unicellular organisms.

Furthermore, researchers found that the new traits could be inherited by the next generations of yeast cells, indicating that prions may play a role in protein-based yeast inheritance and adaptations. The study’s findings on protein-based inheritance indicates that prions may even play a role in the evolution of yeasts.

More researchers will surely follow to further investigate how these findings may apply to people. Nevertheless, the Stanford study’s exciting findings indicate a greater potential understanding of the proteins that have struck fear into the hearts of the people who have heard of them and confusion among those who have not.

<Edward Kim/ Oxford Academy 12th Grade>