Prion Diseases: Their Environmental Impact and Strategies Against Them

Amanda Ivester

Health 4310
Dr. Falta

April 11th, 2014

In 1952, when Hershey and Chase proved that DNA was the heritable genetic material,
there was little room left for debate in the scientific community upon how disease-causing agents
were able to reproduce and infect other organisms (DNA: The Genetic Material ). However,
this era of understanding only lasted for roughly ten years. By 1961 a new agent of disease with
no recognizable nucleic acids had begun to emerge (Belay, 2005). This new agent of disease was
eventually christened prion in 1982 by Prusiner in order to highlight the importance of its
protein component. (Belay, 2005). Prions, although still uncommon, are one of the most deadly
agents of disease that the scientific community is currently aware of, with a 100% fatality rate
once infected (Hayes, 2010). Prions greatly impact human health and the environment in ways
and most individuals are not aware of some of the interventions being taken against this new and
deadly agent of disease.
Prions are abnormal versions of a protein typically found on neurons. The most important
difference in prions and normal neuron proteins lies in their shapes. Ordinarily these neuron
proteins are found in the form of an alpha helix, which looks like a spiral. Prions are composed
of approximately 50% beta pleated sheets, which look similar to a folded sheet of paper. One of
the basic tenants of biology is that shape determines function, thus, if you change the shape of
something, you impair or change its function. Prions, through a currently unknown mechanism,
tell normal neuron proteins to misfold and alter their shape from alpha helix to a mix of alpha
helix and beta pleated sheets. Subsequently, any and all new neuron proteins that are produced
are produced in the new incorrect shape, wreaking havoc on the infected organism (Belay, 2005).
These prion diseases exist in both animal and human forms and act similarly. The animal
form of prion disease is bovine spongiform encephalopathy (BSE) which is more colloquially
known as mad cow disease while the human form of prion disease is known as Creutzfeldt-Jakob

disease (vCJD) (The Basics of Mad Cow Disease). Because these two diseases are different
only in name and infected organism, they are able to be transmitted from animal to human and
human to human. Fortunately, prion diseases are both extremely rare and difficult to transmit. To
date the only known way to spread prion diseases is through contact with infected central
nervous system tissue or contact with the prion itself (Information about Prion disease).
The major symptom of prion disease in both humans and animals is neurological
degeneration. In animals this neurological degeneration presents itself as inability to walk, poor
balance, and aggression (The Basics of Mad Cow Disease). In humans this neurological
degeneration presents similarly to dementia and Alzheimers disease. Humans present with mood
disturbances, personality changes, communication difficulties, memory loss, balance problems,
and visual issues (Information about Prion Disease).
Knowing how rare these prion diseases are, it is easy to ask why one should even care at
all. The answer to that question is more fascinating and terrifying than anyone could have ever
anticipated. The reason that I personally care about prions at all is the fact that they are all but
indestructible. Although prions are proteins, they are resistant to proteases, which are enzymes
that break down proteins. Prions are also resistant to cooking and standard chemical sterilization
procedures. In several cases prions have still been present even after soaking exposed medical
equipment in bleach. Presently the only way to partly destroy prions is through autoclaving,
which is heating the equipment under high pressures and temperatures (Belay, 2005). Not only
are prions virtually indestructible, they are also difficult to detect. In humans brain abnormalities
can only be detected during the advanced stages of vCJD through magnetic resonance imaging
(MRI) and even that does not confirm the disease, just the abnormality (The Basics of Mad
Cow Disease). Currently the only way to officially confirm a prion disease is after death

through analysis of the central nervous system tissue (Hayes, 2013). So, now we are faced with a
rare but difficult to detect disease that is almost impossible to destroy and can be transmitted
across species. Knowing all of this, it becomes far clearer why one should care about prions at
all.
Although still relatively rare, prions do have a devastating effect when they take hold.
Prions are becoming a tangible and real health concern, with creasing numbers of outbreaks as
the years go by. The following examples are just some of the outbreaks of prion disease in order
to illustrate that prion diseases are in fact a significant environmental health concern. vCJD can
be iatrogenic, meaning the disease is transmitted through the course of medical intervention. The
first case of iatrogenic vCJD occurred in 1974 through a corneal graft and there have been three
additional confirmed cases of vCJD linked to corneal transplant across the world (Belay, 2005).
In 1977 there were two more reported deaths linked to vCJD due to use of contaminated
electrodes during an EEG. The electrodes, however, had been cleaned using normal chemical
sterilization procedures. Over two years later researchers demonstrated that the electrodes still
carried the prions by using them on a chimpanzee who eventually exhibited symptoms of prion
disease 18 months after the use of the electrodes (Belay, 2005). There have also been reports of
vCJD being transmitted through pituitary-derived human growth hormone. During a program in
1985, 7700 people were given pituitary-derived human growth hormone. After a follow up of
6,272 of those people, it was reported that 26 of these individuals had developed vCJD by April
2004 (Belay, 2005). One of the largest cases BSE occurred in the United Kingdom beginning in
1986 and involving roughly 750,000 cattle. By 1996 it was discovered that BSE was
transmissible to humans and as such many safety measures were implemented in order to prevent
BSE (Belay, 2005). The most recent potential case of vCJD occurred among 15 patients at three

different hospitals in New Hampshire, Massachusetts, and Connecticut. The patients were
exposed to the vCJD by equipment that was shared between the hospitals. Because there is no
way to screen for prion diseases, these patients will be forced to wait upwards of 20 years before
they will have any idea if they have contracted the deadly prion disease vCJD (Hayes, 2013).
Although there are only about 200 documented cases of prion diseases in the US
annually, they are an incredibly important agent of disease to be concerned about if for no other
reason than the simple fact that the scientific community knows almost nothing about them
(Hayes, 2013). Furthermore, prion diseases are one of the few almost undetectable and
untreatable diseases. Couple that with the fact that the prions are enduring and we have no
reliable method to destroy them, we suddenly have a disease that is capable of wreaking havoc in
hefty amounts. Knowing this, one must question what plans the medical and scientific
community have for limiting the spread of these deadly and dangerous diseases.
Currently most, if not all, of the actions and strategies against prion diseases deal with
preventing the spread of BSE from bovine to bovine and bovine to human. Because iatrogenic
prion diseases are still quite rare, not much research is being done to develop ways to test for and
treat prion diseases. There is also minimal research being done to find a cost efficient and
effective way to sanitize medical equipment in the event it does come in contact with prion
diseases. Unless iatrogenic prion diseases become more prevalent, the most concentrated efforts
against prion disease will remain largely with the animal husbandry industry.
At the international level, the World Health Organization (WHO) has recommended
several policies to prevent the spread of BSE. The number one recommendation the WHO gives
to prevent the spread of BSE is to simply stop feeding animals they are displaying possible signs
of BSE to other animals (Greger, 2003). The WHO also recommended that countries establish

adequate testing and surveillance for BSE and stop feeding bovine brains, eyes, spinal cords,
intestines, or any other tissue likely to contain BSE to humans or livestock (Greger, 2003). For
many countries in Europe these WHO recommendations have become actual law, in large part
due to the fact that the most historic BSE outbreaks have been in European countries. Until very
recently BSE was almost unheard of in the United States and therefore the United States is
severely behind many European countries in regards to BSE prevention.
Although the US isnt entirely following the WHO recommendations against BSE, there
are things being done to prevent BSE. Both the Food and Drug Administration (FDA) and the
United States Department of Agriculture (USDA) have webpages dedicated to BSE and detailing
strategies being used to combat BSE and keep the population safe. Since 1997 the FDA has
banned the use of the majority of cow parts in the making of bovine feed and in 2009 these same
high risk cow parts were banned from any animal feed, including pet foods (All About BSE).
The FDA and USDA work together to ensure that any cows or cow products considered at high
risk for BSE are not imported into the United States. The FDA has also entirely banned the use of
neural tissue and other high-risk cow parts from any food made for human consumption.
Furthermore, any cows that demonstrate inability to walk or other symptoms of prion disease are
also banned from being used in food (All About BSE). According to the FDA since these
measures have been implemented, only three cows have had documented cases of BSE, the last
of which was in 2006 (All About BSE)
For now the FDA and USDA measures against BSE seem to be working quite well;
however, there is little data on the forms of prion disease that affect animals other than livestock.
Currently it is legal in the US to use deer and elk known to be infected with chronic wasting
disease (another form of prion disease) in animal feed for pigs and chickens. I believe it is in

these areas that the US has much room for improvement and research. It is currently believed
that prion diseases are transmissible between animals and humans freely. Around the world there
have been documented cases of prion diseases in bovines, humans, cats, deer, and elk (Greger,
2003). Little research has been done into whether or not pigs and chickens can develop prion
diseases after eating feed made from contaminated deer and elk, but there is great concern that
they may indeed be carriers of prion diseases and pass them along to other animals and even
humans (Greger, 2003).
Prion diseases may be rare but that doesnt mean they are not dangerous. Prions are
virtually indestructible and prion diseases have always been 100% fatal in all documented cases,
human or animal. Internationally speaking a great deal has been done to combat prion diseases in
animals but at national levels, most particularly in the United States, there is still much to be
done. Ideally in the years to come there will be more research into both animal and iatrogenic
prion diseases and the United States while have better preventative measures against this deadly
agent of disease.

References
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Belay, E. D., & Schonberger, L. B. (2005). The Public Health Impact Of Prion Diseases. Annual
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The Basics of Mad Cow Disease. (n.d.). WebMD. Retrieved February 12, 2014, from
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