Prion Disease Loop

After our class on Prion diseases, I was interested in finding out more about potential solutions for preventing or stopping chronic wasting disease. Because Prion proteins can manipulate normal proteins into changing their form to fit with the Prion, it is difficult to find a way to inhibit the stacking and changing of protein shape.

In a UC San Diego article that I read, scientists had found a loop in the protein, signifying a possible way to stop the conversion of normal proteins into prion proteins. They figured this out through testing elk prions (the chronic wasting disease also occurs in deer, aside from cows, sheep, goats, and humans) in a human-like scenario–on mice. Interestingly, though humans are susceptible to “mad cow” disease through the ingestion of sick cow brain, they are not susceptible to the equivalent in deer. The reasoning behind this is a “loop” in normal proteins, which cause human proteins and elk prions to be incompatible.

The researchers are excited about the prospect of finding a potential cure for chronic wasting diseases. Currently there are five forms of human CWD: Creutzfeldt-Jakob Disease (CJD), Variant Creutzfeldt-Jakob Disease (vCJD), Gerstmann-Straussler-Scheinker Syndrome, Fatal Familial Insomnia, and Kuru. Though these diseases are rare, they are 100% fatal and as a result deserve more research for prevention or antidote.

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