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Essay on prion disease
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Essay on prion disease
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Prevention and Safeguard Measures For Bovine Spongiform Encephalopathy (BSE).
Abstract
Bovine Spongiform Encephalopathy (BSE), commonly referred to as mad cow disease, is a disease that damages the central nervous system and results to death. This disease is caused by abnormal proteins, or prions, and directly kills nervous tissues, thus is directly linked to the spinal cord and brain. The United States Department of Agriculture and the United States Food and Drug Administrations have set safeguards and firewalls to prevent BSE-contaminated beef from entering into the human food supply. Human consumption of BSE- contaminated beef results to variant Creutzfedt-Jakob disease (vCJD), which is also fatal. So far, there are no cures for BSE, or for vCJD, but only treatments to help stabilize the symptoms of the disease as the disease progresses.
Bovine Spongiform Encephalopathy (BSE), also known as mad cow disease, is a slowly progressive, degenerative, fatal disease that damages the central nervous system of adult cattle. It is universally accepted among the scientific community that the cause of BSE is from infectious forms of prions, which is a type of protein that is found in animals and is a type of transmissible spongiform encephalopathy (TSE). The exact cause of BSE is not known. In cattle that have BSE, these abnormal prions initially occur in the small intestines and tonsils, and are found in the brain, spinal cord, and other nervous tissues of animals in later stages of this disease (USFDA/CFSAN, 2005). These abnormal prions trigger chain reactions through all the other proteins in the brain and cause those proteins to change into the abnormal prions, thus damaging the brain and the result is death (Massachusetts Dep...
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...h. July 20, 2005: www.mass.gov/dph/cdc/factsheets/madcow.htm
Spengler, R. (2004, February). Mad Cow Disease. Yahoo Health. July 20, 2005:
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Prevention and Safeguard Measures for BSE 7
About BSE in Products Regulated by FDA’s Center for Food Safety and Applied Nutrition (CFSAN). U.S. Food and Drug Administration. July 20, 2005: www.cfsan.fda.gov/~comm/bsefaq.html
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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.
Creutzfeldt-Jakob is known as a prion disease. Prion is a protein that occurs normally inside the brain, however
Chronic Wasting Disease (CWD) is a fatal neuro-degenerative, transmissible spongiform encephalopathy (TSE) of the family Cervidae (Hamir, et.al., 2006). The family Cervidae includes mule deer, Odocolileus hemionus, white-tailed deer, Odocolileus virginianus, Rocky Mountain elk, Cervus elaphus nelsoni, and moose, Alces alces shirasi, among others (Sigurdon & Aguzzi, 2007). CWD is a prion disease, meaning it is a protein caused infection, that occurs naturally in the deer family (Song & Lawson, 2009). This protein is suspected to be an abnormal isoform (PrPSc) of the naturally occurring host prion protein (PrPC) (Blanchong, et. Al., 2009). Bovine spongiform encephalopathy (BSE), mad cow disease, is a similar prion effecting cattle as CWD affects Cervidae. Although, scientists are not sure of transmission route it is suspected that CWD is transmissible and infectious through direct contact with infected individuals or through environmental contamination (Song & Lawson, 2009). Tests have been performed showing susceptibility of altered mice to oral transmission, mimicking the suspected route of entry, and the incubation appears slower but lasts longer with oral infection (Trifilo, et.al., 2007). The approximate time from the initial infection to death is three years.
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).
Normally, cows in Northern Europe in places such as Denmark live normal lives simply grazing on grass, and existing. However, there have been recent changes that have disrupted this normal activity. Generally the bluetongue virus (spread by Culicoides imicola, a biting midge) has been confined to Southern Europe and other places around the Mediterranean. But with the increase in temperature throughout the area, the midge has been allowed to migrate northward. This new pest is a nuisance and causes lots of difficulties to farmers in the area. When a cow contracts this disease, they usually also receive oral ulcers, salivation, stiffness, fever and eventually the inevitable- death (Merck Veterinary Manual NP). Because of the increase in temperature, midges have spread around the globe infecting livestock and creating terrible trouble for many farmers.
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).
HIPPA COW Security Administrative Safeguard Policy/Procedure Workgroup. (2006). Contingency Planning (Draft, Version 8). Wisconsin: HIPAA Collaborative of Wisconsin.
US Department of Agriculture which was renamed the FDA in 1930. The responsibilities of the
Tirrel, Meg. "FDA Delays Food Safety Regulations." Claim Journal. N.p., 20 Dec 2013. Web. 23 Jan 2014.
On January 4, 2011 President Obama signed into law The Food Safety Modernization Act (FSMA). This law has shed new light on the safety and security of our food supply. The last update to the food safety laws in the United States was in 1938. The food safety modernization act pays special attention at trying to modernize the food safety policies in the United States in hopes to prevent problems and concerns before they happen. As we all know, most of our food comes from overseas or sometimes from your neighboring state. The food products travel by car, truck, airplane, boat, or even train. We are all very happy to be receiving our bananas from Costa Rica and all of our other fresh fruits and vegetables that are imported into the United States, but we never stop to think about what pathogens are contaminating our produce and other foods on the way over and if they are safe for us to eat. In an article by Neal Fortin, he states that the law also gives the FDA new standards to hold imported foods to the domestic food standards and it also encourages the FDA to establish and develo...
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.
U.S. Food and Drug Administration. “CFR -- Code of Federal Regulations Title 21” (21CFR101.9). U.S. Food and Drug Administration. U.S. Department of Health & Human Services, 1 Apr. 2013. Web. 21 Mar. 2014.
Cows are naturally very gentle and calm creatures. These smart and sweet natured animals have been known to go to great lengths to escape slaughterhouses. More than forty-one million of these sensitive animals suffer and die a painful death each year in the United States. When cows are still very young they are burned with hot irons, there testicles are torn or cut off, all without painkillers. Most beef cattle are born in one state, live in another, and are slaughtered in another. The cows who survive the gruesome transportation process are shot in the head with a bolt gun, hung upside down by there legs, and taken onto the killing floor where there throats
To begin, vegetarians argue that a meatless diet improves one’s health. Granted that this statement is widely accepted because plant based foods are proven to have a smaller fat content than meat based foods, “vegetarianism is [not] ‘a vaccine against all health problems,’ warns Carol Coughlin, a registered dietitian at the American Dietetic Association” (“Vegetarianism” Issues and Controversies). In fact, the lack of meat in a diet arouses many health issues. For example, vitamin B12 deficiency leads to damaging of nerve endings. This deficiency surfaces in vegetarian diets because vitamin B12 is not present in plants; on the other hand, animals are a source of vitamin B12 because they can consume the microorganisms that produce vitamin B12 (Mangels). Another argument posed by vegetarians is that meat found in supermarkets are ridden with bacteria. However, according to Steven Kopperud, president of the Animal Industry Foundation, “‘salmonella is found on all foods, including fruits and vegetables,’” and “‘E. coli contamination is more common in water ...