Friday, September 9, 2016
From scrapie to Bovine Spongiform Encephalopathy, or better known as Mad Cow Disease, prion diseases have interesting characteristics. According to the Centers for Disease Control and Prevention (CDC), a prion disease is a rare neurodegenerative disorder that is caused by "prions", which are pathogenic agents that can be transmitted and promote abnormal folding of proteins. These diseases can affect both humans and animals. One prion disease that I find to be very interesting is Fatal Familial Insomnia. This specific prion disease causes insomnia that gets worse and causes various symptoms to get worse, such as hyperventilation or the loss of control over your bodies movements, until the individual dies. This disease, like all prion diseases, has no cure. This is because the prion protein, which is caused by a mutation in the PRNP gene, is still a mystery when it comes to its' function. According to the National Center for Advancing Translational Sciences, scientists believe that the protein might play a key role in the brain since the mutation of the gene causes the protein to fold incorrectly and form clumps. These clumps will accumulate in the brain and destroy neurons, which leads to small holes in the brain. Researchers are actively working to find a cure, but unlike most viruses, prion diseases lack DNA or RNA. Prions are made of protein and a treatment or cure for this kind of disease has yet to be found.
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The life expectancy is not very long, somewhere from a few months to couple years.This disease also happens to be an autosomal dominant trait, which means that it can be passed on to your children. Very interesting but scary at the same time.
ReplyDeletePrion diseases are very interesting and also something that I may lose sleep over because it can be inherited or spontaneous. After reading this, I looked up other prion diseases and I found an interesting case with a prion disease known as kuru. Kuru is a famous epidemic where a prion disease was passed from person to person by cannibalism in Papua, New Guinea. The people of New Guinea who ate brains of dead people as part of a funeral ritual. The infectious protein contaminated the brain tissue and incubated in there for a while before showing symptoms of the prion disease.
ReplyDeleteI was wondering the other day when I heard this in one of my classes, can the patient with Fatal Familial Insomnia be forced into a coma? I'm not sure how that works, but I've never heard of that happening to anyone. It probably wouldn't do much in terms of time because we still haven't found a cure, but it seems like it's worth a thought.
ReplyDeletePrion research is an interesting subject. It is unlikely that people contract a prion disease, but with incubation times that can last a decade it is also hard to track exposure. Prions are also incredibly hardy, able to last years in the soil (as seen in cases of Mad Cow disease) and one of few organisms that will not be destroyed in an autoclave. To get rid of prions it is often necessary to use both chemical exposure and autoclaving on instruments, or extreme heat in the case of organic tissue. Some of the attributes that help prions survive unhospitable environments likely make it hard to treat, so I would be interested in seeing the approach scientist take in developing treatments for diseases caused by or in conjunction with prion infection.
ReplyDeletePlease link to your sources...prion diseases are very interesting, but you haven't offered the reader a means of fact-checking or further reading.
ReplyDelete