GRT task 2 Essay

A prion disease is defined when an altered form of a protein is capable of catalyzing a near identical change in another copy of the stated protein.These can be transmitted via an altercation of the protein as it is generated, or through the interactions of normal proteins with the mutated variety. Many yeast, fungal, and human prion diseases form amyloids. These fibers are characterized by a β-sheet rich conformation. Generally these sheets are highly repetitive, and therefore have a high propensity to self-assemble and perpetuate throughout the cell. Generally these areas of high repetition, or prion forming domains (PFDs), are intrinsically disordered and are rich in glutamine and asparagine. According to a study done by Ricardo Sant’Anna et al. four yeast proteins contain protein domains fitting this characterization; Mot3, Sup35, Swi1, and Ure2; have a high likelihood of aggregating into β-sheet rich amyloid fibrils. Using infrared spectroscopy this experiment was able to show that these proteins convert from the normal disordered coil structure into one that has a rich B-sheet configuration when placed under stress. Upon further investigation these amyloid structures were displaying a cross-B structure that could build into a larger amyloid fiber. This experiment

     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).

BSE can be transmitted from one human to another through cannibalism or through transplantation of infected tissue. Consequently, certain human blood products and blood donations are not accepted from people who have lived in areas of the world where BSE outbreaks have occurred in cattle. BSE has been shown to be transmissible by blood transfusion in an experimental model using sheep. The U.K. surveillance unit for vCJD has also reported three cases of vCJD that occurred as a result of blood transfusion

2Jordanne Rice21944377As a Veterinary technician, you may encounter the many diseases that are carried by largeand small animals. Having knowledge of such diseases can prevent the spread to other animals and decrease any chance of human health endangerment. Further paragraphs will include the etiology, signs and symptoms, diagnostic tools, treatment protocols, and prevention and control programs for a handful of the many diseases that animals can encounter. Bovine Spongiform Encephalopathy (BSE) is a neurological disorder that affects cattle and that results from infection by a transmissible agent called a prion (1A). It is a contagious and infectious disease. It is also called mad cow disease. The term spongiform comes from the way the brain

Creutzfeldt-Jakob disease is a fatal neurodegenerative disease that causes rapid degeneration of the cerebral cortex (the outer layer of tissue surrounding the brain). It is closely related to bovine spongiform encephalopathy (BSE) or “mad cow disease” in its pathology. Creutzfeldt-Jakob disease is often called the human version of BSE and is therefore assumed that it has crossed the species barrier from cattle to man. In the early stages of Creutzfeldt-Jakob disease, it is found that patients have a failing memory, behaviour changes, impaired coordination and vision problems. As the disease progresses into the later stages, the mental deterioration becomes severe. The patients experience uncontrollable movements, blindness, and weakness and

This accounts for about 5% - 10% of cases in the United States. The last category of acquiring CJD is infectious CJD which occurs when an individual is exposed to an external source of prion protein. Common outside sources which may cause infectious CJD are medical procedures involving surgical instruments, growth hormone from human sources and certain transplanted human tissues. Another cause is ingestion of meat or other products from cattle infected with bovine spongiform encephalopathy (also known as mad cow disease). In the mid 1990’s scientists traced this type of CJD from cattle that were fed a diet comprised of processed brain tissue from other animals to individuals who consumed the beef. However, since its first description, less than 1% of cases were identified by acquiring the disease through exposure (Gozke et al. 2008). Below is a chart demonstrating the numbers of individuals diagnosed with CJD through the three categories described above in several different countries (“BSE Info”, 2007):

Bovine Spongiform Encephalopathy is more commonly known to humans as Mad Cow Disease or BSE. BSE 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).

The word BSE is short but it stands for a disease with a long name, bovine spongiform encephalopathy. "Bovine" means that the disease affects cows, "spongiform" refers to the way the brain from a sick cow looks spongy under a microscope, and "encephalopathy" indicates that it is a disease of the brain. BSE is a progressive neurologic disease of cows. Research shows that the first probable cases of BSE in cows occurred during the 1970 's with two cases of BSE being identified in 1986. BSE possibly originated as a result of feeding cattle meat-and-bone meal that contained BSE-infected products from a spontaneously occurring case of BSE or scrapie-infected sheep products. Scrapie is a prion disease of sheep. Similar diseases such as Chronic wasting disease which is in both deer and elk populations is devastating the hunting and the reproduction of deer. Other similar disease such a the Creutzfeldt-Jakob disease which occurs in the human body system. That is pretty scary for us humans. We have got to find a way to get this prion settled down and hopefully I can show you the way the scientist plan to get rid or at least slow it down. There is strong evidence and general agreement that the outbreak was then amplified and spread throughout the United Kingdom cattle industry by feeding rendered, prion-infected, bovine meat-and-bone meal to young calves. A lot of people wander what does it to the consumption of the meat does it ruin it or is it ok to eat? What does the meat that

BSE is a kind of transmissible spongiform encephalopathy (TSE). TSEs can emerge in creatures that convey an allele which causes beforehand ordinary protein particles to bend independent from anyone else from an alpha helix course of action to a beta sheet, which

There are a number of other proteins that display prion-like behaviour despite they do not fit in the previous definitions. These functional prion-like proteins differ from pathogenic prions and prionoids in two important aspects: First, the conversion to the aggregated state is regulated by a physiological signal, and second, the prion-like conformation exerts a defined novel function.

The most common is route is ingestion of diseased tissue (“John Hopkins Medicine: Prion Diseases”, 2016). This is seen in bovine spongiform encephalopathy (Mad Cow) in the 1980’s in the United Kingdom when cows were fed diseased cow brain tissue and then it continued to spread among cows and eventually spread to humans consuming diseased cow meat. This is what is called variant Creutzfeldt-Jakob disease. There is a different type of Creutzfeldt-Jakob disease is inherited. This is called familial CJD. The most rare variation of CJD is called sporadic. This is were the disease has a sudden and unexplainable onset and usually affects individuals in their 60s. The other variation of prion disease that is found in humans is Kuru. Kuru is a prion disease that was found in tribes that practice cannibalism specifically in eating raw human brain matter. This is an extremely rare disease and has not been discovered in places that do not practice

Transmissible Spongiform Encephalopathies (TSEs), sometimes referred to as prion diseases, are a series of rare degenerative brain disorders portrayed by miniature holes that makes the brain the same appearance as a sponge. TSEs are caused by an unusual version of a protein called a proteinaceous infectious particle, also known as a prion. Prions can occur in a normal form, which does not cause harm to the body’s cells, and in an infectious form, which causes substantial harm to the body’s cells. Both forms of the prion are almost identical, but the harmful form has a distinctive folded shape. TSEs has the ability to occur sporadically, hereditary, or transmitted from an infected individual. Sporadic TSEs can develop with no cause. In some

Previous studies have shown that different proteins contain short identical sequences in their secondary structures that have completely different functions and folding mechanisms. It has also been shown that these same sequences can be induced to form an α helix, β strand, or β sheet structure depending on the context. These findings have suggested that chameleon sequences have a sense of structural plasticity which can pose a problem for protein secondary structure prediction. While previous research has only focused on the notion of identical sequences within an α helix in one protein whereas a different protein has the same identical sequence but instead of an α helix, the sequence is within a β strand; Guo et al. delve deeper into the notion that there is also the possibility of these sequences being found within a β sheet or β hairpin conformation. To distinguish between the two, Guo et al. introduced the terms chameleon-HS and chameleon-HE. Chameleon-HS refers to the sequences with helix and strand structures whereas chameleon-HE refers to the sequences with helix and sheet/hairpin structures.

provide a place for the bases to attach. The bases determine the order of amino acids and thus

Prion diseases or transmissible spongiform encephalopathies (TSEs) are a family of rare progressive neurodegenerative disorders that affect both humans and animals. They are distinguished by long incubation periods, characteristic spongiform changes associated with neuronal loss, and a failure to induce inflammatory response. The proteinaceous infectious particles, prion was identified by an American neurologist Stanley B. Prusiner and colleagues in 1982derived from the words protein and infection (Stanley B. Prusiner -Autobiography). Prions are known to Creutzfeldt - Jakob disease (CJD), Gerstmann–Sträussler–Scheinker syndrome and Fatal Familiar Insomnia in humans. In mammals they cause "mad cow disease" in cattle. Prion diseases affect