Prion

Mad Cow Disease Bovine spongiform encephalopathy (BSE), better know as Mad cow disease is a relatively new disease. Most sources state that BSE first showed up in Great Britain in 1986 [Dealler p.5] but some say it popped up in 1985 [Greger p.1]. However the official notification was not until 21 June, 1988 [Dealler stats. p.1]. Spongiform encephalopathies are invariable fatal neurodegenerative diseases and there is no treatment nor is there a cure for this disease [Greger p.1]. The recent scare

Variant Creutzfeldt-Jakob disease is an infectious and transmittable form of a prion, although extremely rare it still becomes highly fatal if infected. This disease can also be classified as a Transmissible Spongiform Encephalopathy (TSE) that is gained through exposure to the Bovine Spongiform Encephalopathy (BSE) from contaminated cattle or meat. As this is not a prominently spread disease around the world and considering it was only recognised in 1996 it has only held 175 cases in the United

individual by gradually destroying their memory, vision, ability to think and move voluntarily and function independently. It is one variety of transmissible spongiform encephalopathies (TSEs) and is categorized under a group of brain disorders known as prion diseases (Kraft, 2014). This disease is very rare affecting about one person in every one million worldwide; in the states there are only about 300 cases a year. CJD interested me as my sister’s god father (my dad’s best friend) recently passed about

to humans. There have been three main theories of the cause of scrapie and its resistant nature to all previous experimental cures. The first theory is that the agent is a virus with unusual characteristics. The second theory is that the agent is a prion, which is an exclusively host-coded protein that is modified to a protease-resistant form after infection. The last theory is that the agent is a virino, a small nucleic acid coated with a host-derived protective protein that allows it to resist scientists’

Prions are infectious protein agents that cause aggregation of the peptide PrPC and form large amyloid fibrils. These fibrils resist proteoysis and can have a detrimental effect of the functionality of cells and ultimately cause their death. (3). Misfolded PrPC will form stable aggregates denoted as PrPSc. PrPC is a soluble protein that is present in all organism whose function is still not totally clear (3). When PrPc converts to PrPSc there is an increase in the number of β sheets in the protein

with diseases such as Mad-Cow disease, Scrapie, and Creutzfeldt–Jakob disease. With well supported evidence it is hypothesized that CWD originated from Scrapie. The causation of CWD is an abnormal form of PrPcwd prion. This prion is a naturally occurring protein found in mammals. Prions lack genome and are highly resistant to degradation. Chronic Wasting Disease can be transmitted by direct contact between the animals and/or on resources such as salt licks. With a team, Chris Janelle, studied

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. In Canada, however, there have been 19 cases of mad-cow disease. This differs considerably

results from infection by an unusual transmissible agent called a prion”. (Center for Disease Control & Prevention, 2015). There are two varieties of Bovine Spongiform Encephalopathy. One is classical and the other is atypical. Contaminated meat-and-bone meal, which comes from infected bovine whose body is used to make bovine feed, transfers to the herd that eats this infected feed product. Known organs that contain this prion are spinal cord, brain and ileum. Atypical happens to bovine that

believe that the infectious agent that causes mad cow disease is an abnormal version of a protein normally found on cell surfaces, called a prion. For reasons still unknown, this protein becomes altered and destroys nervous system tissue (brain and spinal cord). There exists strong epidemiologic and laboratory evidence for a causal association between a new human prion disease called variant Creutzfeldt-Jakob disease (vCJD) that was first reported from the United Kingdom in 1996 and the BSE outbreak in

originated from scrapie or Endemic Spongiform Encephalopathy, which is a disease that has affected mostly sheep and goats. BSE is an illness that attacks the brain and spinal cord of adult cattle due to an infection by a transmissible agent known as a prion. Once affected by BSE cattle began to develop strange behavior such as aggression, lack of coordination with the inability to stand or walk, and abnormal posture; hence the name Mad Cow Disease (1). Why BSE Should Be Eradicated- -BSE is a fatal disease-