Ashley's Biggest Takeaways
- Prion proteins, when misfolded, are infectious and can cause disease in humans and animals.
- The exact mechanism by which prions get converted from their normal cellular form to the infectious form is unknown, but a key area of research.
- The most common route of prion disease transmission is oral (i.e., through the handling or eating of contaminated meat or tissues).
- Prions can also be transmitted through blood transfusions or organ transplant.
- Prion diseases can be difficult to distinguish from one another due to similar pathology and protein folding.
- Research indicates that once inside the body, prions hijack immune cells and accumulate in lymphoid tissues before spreading to the central nervous system.
- Prion accumulation varies between diseases and tissues, affecting disease duration and clinical phase.
- Early symptoms of prion diseases can be non-specific and difficult to distinguish from other illnesses, such as flu or COVID-19.
Featured Quotes:
“It's something that's been occupying our minds for decades. In fact, there have been several Nobel prizes awarded in this field, and still amongst the field, we still argue about what a prion actually is.""But I guess we're slowly reaching consensus in this, and I say slowly because there will always be somebody who disagrees with us, and science is ever correcting, etc. But we really consider now prions to be infectious proteins."
"So, every cell in our body expresses the normal cellular form of the prion protein. And we really don't know what this protein does. It's highly conserved in mammals, expressed on most cells in our body. Some cells express it really highly, such as neurons."
"During prion disease, this protein starts to abnormally fold. So, normally, the protein is expressed in a predominantly alpha helical form. It has these kind of curly tubes. During prion disease, it starts to misfold and becomes more of a beta pleated sheet. And when that happens, it confers some really interesting biochemical and biological properties to that protein"
"Whereas the normal cellular form of the protein is expressed on the cell membrane—it's soluble in detergents, it's relatively easily digested by proteases, etc.—the abnormally folded form, which we find during prion disease now forms aggregates, often in the extracellular environment. It also becomes very resistant now to standard decontamination procedures. They’re resistant to proteases, but most importantly, it becomes infectious."
"By that I mean that if, we were to inject some of those proteins into a specific animal or person, those proteins can, by some unknown mechanism, convert the normal cellular form into the infectious form. So, you get amplification of those prion proteins, and they can transmit disease, and ultimately, if left unchecked, lead to severe damage of the neurons in the brain of those animals or people."
"As microbiologists and scientists, we're interested in natural disease, and that's the area that I've been studying for at least 2 decades now: how do the prions go from the site of infection to the brain where they cause disease?"
"A predominant natural route of transmission is the oral route. So, by ingesting or eating infectious prions."
"Prion disease is a neurological disease in the CNS. It can cause extensive damage to neurons in certain regions of the brain. We've been really interested in how do those prions go from the gut or the intestine to the brain, where they eventually cause disease."
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