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essay about prion diseases
essay about prion diseases
mad cow disease 1985
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Mad Cow Disease: A Cause for Fear?
Abstract:
Mad Cow Disease, a disorder well known and well feared, is not as deadly as most
people believe it to be. In fact, most people know little of this disease and what little they know
usually turn out to be false facts. MCD is a prion-based disease where an infected protein converts
healthy proteins into the infectious state. There is no cure and the disease is fatal but to this year, there
have been little over 150 cases of the human version of the disease, variant Creutzfeldt - Jakob disease,
world-wide. Thus, MCD does not deserve the fear and attention that it commands.
Although the name, Mad Cow Disease is instantly recognizable by everyone today, few people
know it for what it actually is. However, no matter how little they know about this disease, everybody
acknowledges the deadly capabilities it possesses. Personally, I find it interesting and feel it only
natural that, as humans, we fear what we do not entirely understand simply because we know what will
happen. It is only natural that we feel threatened at the first sign of danger. Thus, it is important to
inform others about Mad Cow Disease so that they will at least know what they fear.
Bovine Spongiform Encephalopathy, commonly known as Mad Cow Disease, first appeared around
1985 in Great Britain as a result of the special animal feed that consisted of ground up sheep fed to the
cattle. Between the years 1992 and 1995, a major BSE outbreak occurred in Great Britain, which caused
the country's beef industry to suffer. Since 2003, the United States have only had three reported
incidents of mad cow disease in humans[8].
A prion is a disease-carrying agent that is composed entirely of proteins. It is the cause of
numerous diseases in mammals, all of which target the brain specifically. Also note that all prion-based
disorders are fatal and that there are currently no known cures for any of them. All known prions
induce the formation of an amyloid fold, in which the protein polymerizes into an aggregate consisting
of tightly packed beta sheets[3]; thus, converting into its infectious state.
BSE is a prion-based disease where the infectious protein causes other prion proteins in the brain to
misfold and change into the infectious state. This
process continues until it forms protein
aggregates, which are large clumps of mis-folded
proteins which in turn form plaque, giving the
appearance of “holes” in the brain. Cows with
this disease show signs including changes in
mental state and abnormalities in posture,
movement and sensation[4].
In the subsequent essay I will discuss and explain the relative function of the Prion protein. The Prion protein, also known as PrPC, ‘’is a membrane-anchored protein with two N-glycosylation sites and, although it is highly expressed in the nervous tissues, its physiological functions have yet to be well established’’ (Coordination Chemistry Reviews). PrPC/PrP is found in healthy brains in this form, and consists of 250 Amino Acids, yet after a simple misfolding in the secondary structure; this can alienate the PrP and forms PrPsc, which is the abnormal form of the Prion protein. The infectious agent PrPsc causes neuropathological changes in the brain, and instantly places the individual under the category of someone with the prion disease. PrPsc forms insoluble fibres and thus cannot be studied well using Nuclear Mass Resonance (NMR), and it is also more resistant to protease digestion. Furthermore, ‘’ The transmissible spongiform encephalopathies (TSEs) arise from conversion of the membrane-bound prion protein from PrPC to PrPSc, the latter being the scrapie form. Examples of the TSEs include mad cow disease, chronic wasting disease in deer and elk, scrapie in goats and sheep, and kuru and Creutzfeldt-Jakob disease in humans’’ (http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2904554/. 2014). The following diagram shows the conversion from PrPc to PrPsc:
Mad Cow Disease, scientifically referred to as (BSE) Bovine Spongiform Encephalopathy, is a disease that affects those humans who eat the meat from infected cows. Mad Cow Disease is one of several fatal brain diseases called (TSE) Transmissible Spongiform Encephalopathy. (USDA) There was evidence of a new illness resembling the sheep disease scrapie. It was technically named BSE but quickly acquired the mad cow tag because of the way infected cattle behave. (CNN) In 1997, there was an award given to Stanley Prusiner, for concluding that a distorted protein called a prion was responsible for Mad Cow Disease, noted the long incubation period made it difficult to distinguish (Bryant). Another name for Mad Cow Disease is the new variant Cruetzfeldt-Jakob Disease (vCJD), similar to the Creutzfeldt-Jakob Disease, which is a deadly brain illness that strikes about one per million per year (USDA) due to genetic or unknown causes while the vCJD is contracted from eating infected cows (USDA). Both CJD and vCJD are so similarly named because of the similar effects from the illness.
Mad cow disease is caused by prions, "weird mutant proteins that are found in brain and spinal tissue"1. Another name for mad cow disease is called bovine spongiform encephalopathy (BSE) and the definition is "a progressive neurological disorder of cattle that results from infection by an unusual transmissible agent called a prion"2. It started from what is called a prion protein then it turned into a pathogenic, and then it starts to damaged the brain of a cattle. There's another name for this disease and it's called Creutzfeldt-Jakob Disease "a form of progressive dementia characterized by loss of nerve cells and degeneration of nerve cell membranes leading to the production of small holes in the brain. It is rare, degenerative, and invariably fatal"3. This disease happen in human causing lapses in the memory, mood swings similar to depression, lack of interest and social withdrawal3. It is said that this disease has no tr...
Creutzfeldt-Jakob is known as a prion disease. Prion is a protein that occurs normally inside the brain, however
Plenty of people love the fact that they can enjoy life normal because their brain functions accordingly. Well not every one has the gift of normality. In this harsh world people are dying of all types of diseases and things that are undetectable or even treatable due to the fact that they have unknown origins. Many of the world’s diseases such as HIV, AIDS, some cases of Multiple Sclerosis and Muscular Dystrophies. Among these fatal and life threatening ailments Creutzfeldt-Jakob disease, other wise known as (CJD), takes precedence.
PrP can occur in two forms- a normal cellular prion protein known as PrPc and a pathogenic misfolded conformer known as PrPsc. The abnormal PrPsc differs from the normal prion protein PrPc in both secondary and tertiary structure. PrPsc is principally rich in Beta sheet contents but PrPc is principally rich in alpha helical contents. Due to this difference of between the isoforms, prions are extremely resistant to certain decontamination systems. The Two tables below outline both human and animal diseases (2).
The origin of CWD has yet to be determined (Sigurdson & Aguzzi, 2007). The infection was first noted in 1967 at a captive mule deer research facility. In 1978 pathologists recognized the TSE type brain lesions, also that CWD presented as a prion disease by the neuronal perikaryonic vacuoles, the accumulation of aggregated prion protein and prion infectivity in the brain. In the late 1970s and early 1980s the infection w...
Although mad-cow disease is always fatal, it is not really much of a worry in the United States. There have only been four cases of mad-cow disease ever recorded in the United States. In every case, the United States Department of Agriculture has intervened and recalled tons of beef, 10,400 lbs. in the first case to be exact, in order to insure that the meat did not reach the plates of United States citizens.
Chronic Wasting Disease is a highly transmissible, deadly neurodegenerative disease that affects cervids in North America (Belay et al., 2004; Saunders et al., 2012). There are only four types of cervid that are known to get this disease which include elk, mule deer, white-tailed deer, and moose (Chronic Wasting Disease Alliance). It has been classified has a transmissible spongiform encephalopathy (TSE), otherwise known as a prion disease (Belay et al., 2004). A prion is an irregular, pathogenic agent that causes abnormal folding of specific proteins called prion proteins. These proteins are mostly located in the brain (Chronic Wasting Disease Alliance). The abnormal folding of this protein causes neurodegenerative diseases in a variety of species including humans, sheep, cattle, and deer (Abrams et al., 2011).
Bovine Spongiform Encephalopathy or Mad Cow Disease (BSE), degenerative brain disorder of cattle. Symptoms in cows include loss of coordination and a typical staggering gait. Affected animals also show signs of senility, for example, lack of interest in their surroundings, the abandonment of routine habits, disinterest in feed and water, or unpredictable behavior. Affected cattle show symptoms when they are three to ten years old.
Prion Disease is an illness that many have not heard about. This is sad because many have died and are dying from this disease that doesn’t yet have a cure. “Prion Disease is a group of conditions that affect the nervous system in humans and animals… these conditions impair brain functions, causing changes in memory, personality, and behavior; a decline in intellectual function (dementia); and abnormal movements particularly difficulty with coordinating movements (ataxia)” (Genetics Home Reference). This is basically the definition of what Prion Disease is and without going into depth it explains how it affects the person that is affected. “In t...
...covered. If it is true that prions don't cause BSE or CJD, then what does? If they do, what can stop a prion – or can they be stopped? A prion is, after all, a type of pathogen that was only recently identified.
Creutzfeldt-Jakob Disease is an uncommon, deteriorating, consistently fatal brain disorder that is caused by prions. The symptoms of CJD are similar of Alzheimer’s but progress much faster. There are three variations of CJD, sporadic, familial, and acquired. All variations affect the brain the same way and have the same result of death. CJD is an untreatable and incurable disease.
Bovine Spongiform Encephalopathy, or BSE, is a degenerative disease affecting the central nervous system of cattle causing the brain to appear as a sponge. The cattle then begin acting abnormally and eventually have to be killed. BSE can be transmitted to humans if they consume raw meat from an infected cow or if one consumes the eyeballs, spinal tissue, or the brain. This disease is known as Creutzfeldt-Jakob disease. Another disease similar to BSE, which is found in sheep, is Scrapie. Scrapie has been around for nearly two hundred years. It is presumed that the Scrapie agent jumped species and moved into cattle when sheep offal, leftover parts of butchered animals, were ground up and used as a protein supplement in cattle feed and the subsequently fed to cattle.
Many people are also afraid of the “unknown”. The unknown could refer to what might happen tomorrow...