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Mad-cow disease

Improving nutrition is essentially a process of encouraging people to make healthful choices that improve their well-being (Wansink, 2005). What happens, however, when we believe contamination, terrorism, or a genetic incidence threatens a part of the food supply Sometimes crises influence the recall, redesign, and communication efforts of individual companies (such as Tylenol, Perrier, Pilgrim s Pride). Others, such as the threat of mad cow disease (bovine spongiform encephalopathy, or BSE) in beef can compromise an entire industry. [Pg.104]

Congressional Research Service (CRS). 2003. Mad cow disease and U.S. beef trade. CRS Report for Congress, www.ers.usda.gov/features/fse/index.htm (accessed July 4, 2007). [Pg.45]

Bovine spongiform encephalopathy (BSE or mad cow disease) is a progressive neurological degenerative disease in cattle. It is caused by a mutated protein called a prion. BSE was first reported in the United Kingdom in 1986. Creutzfeldt-Jakob disease (CJD) is a rare disease that occurs in humans. Evidence to date indicates it is possible for humans to acquire CJD after consuming BSE-contaminated cattle products. [Pg.344]

Encephalopathies have been known in animals for many years, e.g. scrapie, which occurs in sheep. Others include spongiform encephalopathy in domestic cats and, more recently, bovine spongiform encephalopathy. The latter developed in the UK in the 1980s symptoms include uncoordinated movement and frenzy (hence mad cow disease). There is some evidence that it occurred in cows after they were fed offal that was prepared from tissues of other cattle. [Pg.414]

Scientific procedures for risk assessment include assessment of risk for human health as well as risk for the environment. A substantial part of the EU risk assessment work was in 1997 delegated to the DG SANCO, in relation to the scandal surrounding BSE (bovine spongiform encephalopathy or mad cow disease ). Risk assessment work not under DG SANCO includes pharmaceuticals, working environment, and health effects caused by lifestyle factors such as diet, smoking, and alcohol consumption (EU 2006f). [Pg.41]

Mad cow disease—Bovine spongiform encephalopathy, or BSE, the form of transmissible spongiform encephalopathy (TSE) found in cattle. It is thought to be spread by consumption of brain tissue and caused by proteins called prions. [Pg.156]

Transmissible spongiform encephalopathies (TSEs)—Brain diseases transmitted from one animal to another. Under a microscope, the brain tissue of animals and people with TSEs resembles a sponge. TSEs include variant Creutzfeldt-Jacob disease (vCJD) in humans, scrapie in sheep and goats, and bovine spongiform encephalopathy (BSE) in cows (mad cow disease). These diseases are spread by consumption of brain tissue and are thought to be caused by prions, a kind of protein. [Pg.161]

Prion diseases have attracted immense attention over the past decade. This attention was prompted, in part, by the outbreak of mad cow disease in the United Kingdom, an outbreak that ultimately involved humans. More recently, the identification of cows with mad cow disease in North America has emphasized the widespread nature of this problem. [Pg.514]

Like any other protein, the molecular structure of the prion is subject to conformational flexibility and to various thermal-induced fluctuations between varying conformational states. However, if these fluctuations permit the PrP conformation to be attained, then this abnormal conformer promotes the widespread conversion of PrP to PrP , leading to the precipitous deposition of the abnormal protein throughout the brain (mirrored by the rapid and relentlessly downhill clinical course). This pathological self-propagating shape conversion of a-helical PrP to P-sheet PrP may in principle be initiated by a seed PrP molecule in the neurotoxic conformation. This explains the transmissibility of prion diseases and accounts for how susceptible humans exposed to beef from an animal with mad cow disease develop variant Creutzfeldt-Jakob disease. [Pg.515]

A misfolded protein appears to be the causative agent of a number of rare degenerative brain diseases in mammals. Perhaps the best known of these is mad cow disease (bovine spongiform encephalopathy, BSE), an outbreak of which made international headlines in the spring of 1996. Related diseases include kuru and Creutzfeldt-Jakob disease in humans, scrapie in sheep, and chronic wasting disease in deer and elk. These diseases are also referred to as spongiform encephalopathies, because the diseased brain frequently becomes riddled with holes (Fig. 1). Typical symptoms include dementia and loss of coordination. The diseases are fatal. [Pg.150]


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