Ben Yeow

HM 310 – Tom Bruce

Research Paper
February 23rd 2009

Bovine Spongiform Encephalopathy (Mad Cow Disease)

On December 23 2003, USDA announced the first known case of BSE in the
United States. The adult Holstein cow was non-ambulatory (“downer” cow) at the time of
slaughter. After USDA Food Safety and Inspection Service (FSIS) took brain tissue
samples, inspected the cow before and after slaughter, the officials released the carcass
for use as food for human consumption. On December 24, FSIS recalled slaughtered beef
from this plant on the same day as the BSE-positive cow. By this time, the recalled beef
had already been shipped and processed further in several establishments in Oregon,
Washington, California, Idaho, and Nevada. Fortunately, as of January 7, USDA
confirmed that none of the meat products had left control of the companies and entered
commercial distribution yet. However, following this event, several countries including
Japan and Korea banned U.S. imported beef. This confirmed case prompted officials to
introduce additional safeguards to reduce the risk of human exposure to BSE on
December 30, which included immediate banning of non-ambulatory animals from
human food supply. Some other additional safeguards include requiring additional
process controls for establishments using advanced meat recovery (AMR) systems,
holding meat from cattle until the BSE test are received with negative results, and
prohibiting the air-injection stunning of cattle.

Bovine Spongiform Encephalopathy is the bovine (cattle) form of a group of

diseases known as transmissible spongiform encephalopathies (TSEs). BSE is
characterized by the appearance in neurons in the brain of affected cattle of vacuoles,
clear holes, which give the brain the appearance of a sponge. TSEs are chronic
degenerative lethal diseases that affect the central nervous system of the infected animal.
They are known to occur in sheep and goats (scrapie), deer and elk (chronic wasting
disease), mink (transmissible mink encephalopathy) and domestic cats (feline spongiform
encephalopathy). TSE result from infection of an unusual transmissible agent called a
prion.

Prions are an unusual breed of infectious agent unlike bacteria, viruses, or

parasites. Stanley Prusiner of UCSF was accredited for discovering prions to be the
causative agents of spongiform encephalopathy. They are essentially just rogue proteins
and nothing else (abnormal variants of proteins that occur naturally in cells). They
contain no nucleic acid (RNA or DNA). They consist of a single molecule containing
about 250 amino acids termed PrP proteins. The difference in these abnormal proteins
from the naturally occurring normal proteins in the body do not lie in their primary
structure (sequence of amino acids), but rather in their folding. PrP proteins are folded in
a way that allows them to resist normal protease degradation. Over time, this leads up to a
build up of PrP proteins, especially in the neuron of the brain cell. Prions are unusual
because unlike all other “life forms”, they contain no genetic instruction (carried by
DNA/RNA) to hand down. Yet amazingly, these abnormal PrP proteins can convert their
normal counterparts into more of these abnormal PrP proteins by directing the refolding
of normal proteins simply by mere contact.

There is increasing evidence that there are multiple strains of BSE: typical BSE
strain (linked to the 1986 outbreak in UK), and two atypical BSE strains (H and L
strains). The typical strain BSE has also been identified to be the most common cause of
BSE outbreak in Canada. It can be prevented by eliminating BSE-contaminated feed. The
typical BSE strain has also be causally linked to the human version of TSE known as
variant Creutzfeldt-Jakob disease (vCJD). Variant CJD in humans is believed to be
caused by consuming beef products contaminated with central nervous tissue (brain and
spinal cord) from cattle infected with BSE. This disease is fatal (usually within 13
months of onset symptoms) and can affect all age groups. It is also very hard to diagnose
the disease until it has nearly ran its course. In the advanced stages of vCJD, brain
abnormalities may be detected by MRI. It is commonly mistaken that cooking beef to the
correct temperature may eliminate mad cow disease. However, prion proteins cannot be
destroyed by heat used to cook food. One of the ways prions can be destroyed is through
incineration at 900ºF for 4 hours, which would render any food inedible at this point.

The first case identified case of BSE occurred in United Kingdom in 1986, where
it caused a large outbreak among cattle. The cause of the outbreak was highly attributed
to cattle feed that contained prions from grounded-up meat of scarpie-infected sheep or
cattle with previously unknown TSE (meat-and-bone meal as a protein source). As a
result, protein supplements made from cattle and sheep offal were banned in the UK in
1988. However, the ban was not strictly enforced until around 1991 to 1992.
Consequently, given the long incubation period of BSE, the epidemic curve did not start
to decrease until late 1993. Later in 1996, the causal link between BSE and vCJD was
established, thus increasing concern among the general public and health officials. In
1997, both the US and Canada implemented a feed ban which prohibited rendered beef
protein in beef feed. Various precautionary measures have been established since the
early 1990s, which include surveillance programs to monitor the presence of BSE in
cattle. New programs and safeguards have also been gradually introduced over the course
of time to help eliminate BSE in cattle and entering the human food supply, such as the
banning the beef of a “downer” cattle as human food supply on December 30 2003
mentioned in the beginning of this article.

Since the peak of the 1993 BSE outbreak in the UK through August 2008, 18
cases have been identified in North America itself (3 cases in US and 15 cases in
Canada). Of the 15 cases in Canada, nine of these BSE-positive cattle were born after the
1997 feed ban implementation. Although BSE is fairly low risk in North America today,
both the Canadian Food Inspection Agency (CFIA) and USDA continue to implement
enhanced feed bans to reduce BSE prevalence in cattle. As of July 2007, CFIA placed a
new and enhanced feed ban into effect. With this new measure in place, the CFIA expects
BSE to be fully eliminated from Canadian cattle herd by about 2017. FDA also issued a
new regulation on enhanced feed bans to go into effect on April 2009 to further
harmonize the BSE feed control measures in the United States with those in Canada.
References

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“Sean Henahan” Mad Cow Disease: The BSE Epidemic in Great Britain.
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“Simon Kenyon, Pat Skinner, and Abigail Borron” BSE – Mad Cow Disease.
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