Prions: What They Are, The Damage They Cause, and Why
People Are Afraid of Them
Introduction: While the general public might not know the word “prions” they is certainly familiar with
one of its variants: Mad Cow Disease. Just this phrase strikes fear in the hearts of both cattleman and
consumers of meat. However, there is so much more to Prions than just this one variant. Prions are
unique for their structure, the diseases they cause and the general sense of medical mystery assssociated
about them.
Defining Prions: Prions are misfolded proteins that characterize several deadly neurodegenerative
diseases. Unlike viruses or bacteria, they do not have nucleic acids (i.e., rna or dna) so there should be no
reason why prions replicate, yet they do.
Diseases Prions Variations: Prions are
responsible for a variety of
neurodegenerative diseases in humans
and cows including Mad Cow disease,
Creutzfeldt–Jakob disease, variant
Creutzfeldt–Jakob disease (vCJD),
Gerstmann-Straussler-Scheinker disease,
kuru, scrapie, fatal familial insomnia and
chronic wasting disease. These diseases
have no cure.
Figure One: A normal prion vs. a diseased prion.
Prions in the News: The most famous case of prions is the Mad Cow Disease outbreak in the United
Kingdom in the 1980s and 1990s. The first reported case of MCD was in 1986 after farmers started using
feed that was accidentally laced with the disease due to unsafe practices.
At its peak in 1992/1993 there were over 100000 cases
of confirmed cases of the disease within the cow
population. In order to stop the spread nearly 4 million
cows were killed. However, the effects of the outbreak
were long-lasting. In the UK 178 people from the
disease, however one in 2,000 people are though to be
carriers of the disease, with scientists still unsure of if
and when the disease will take hold in its victims.
Figure 1: Chart of Deaths Associated with Prions in by Age
In the U.S.: Although the US has banned the import of beef from the
U.K. after the outbreak of the 1990’s, there have still been 6 reported
outbreaks of prion related disease in the United States.
Conclusion: Prions are a fascinating set of diseases that have no
cure and inspire fear in the general public.
More money and time
should be spent researching them to alleviate public fears and to
better understand this mysterious category of disease.