Taking a Look at Prions

Proteinaceous Infectious Particles, commonly known as Prions, are extremely rare misfolds of the protein PrPc, which cause fatally neurodegenerative diseases, and are theorized to be infectious only by the protein itself (U.S National Library of Medicine, 1998). This “protein-only theory” is still heavily debated today, as some scientists deny the theory, and there isn’t a significant amount of evidence on each side to qualify the theory or disprove it (Soto, C. 2011). The base “Prion” protein is encoded in the gene PRNP, while being non-infectious. Prions are most commonly found in human prion diseases, but they can also be in other animals in the form of Mad Cow Disease and Chronic Wasting Disease, classified as Bovine Spongiform Encephalopathies

     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).

In bovine spongiform encephalopathy (BSE), the disease is caused by the misfolding of proteins that cause proteins and peptides to develop a fibrillary structure. The PrPc is a correctly folded prion and the misfolded form is called PrPSc. BSE occurs when the normal PrPc come into contact with the toxic PrPSc and the normal prion takes on the shape of the PrPSc. The normal chaperones are unable to convert the PrPSc back to the normal form. The PrPSc now takes on the role of chaperone and the conversion of PrPc prions continue over and over. PrPSc, now being hydrophobic avoids the water of the inner cell and begin to accumulate and form plaques along the neuronal cell membranes. The aggregation of the prions on the cell membrane eventually lead to cell death which produces the sponge-like appearance in the brain of cattle infected with BSE (Thompson, 2014).

Variant Creutzfeldt-Jakob disease (vCJD) is a rare and fatal human neurodegenerative condition falling under the category of Transmissible Spongiform Encephalopathy (TSE) because of having characteristic of spongy degeneration of the brain that it causes and its ability to be transmitted. First it was described in the United Kingdom in March 1996 and it has been connected with exposure to a TSE of cattle known as Bovine Spongiform Encephalopathy (BSE) sometimes called Classical BSE, having been reported first in 1986 in the United Kingdom.

Abnormal changes happen in the brain, and it appears as a sponge-like holes and gaps, causing deterioration in the areas of the brain. This can turn into spongiform degeneration that results in the progressive neurological and neuromuscular symptoms that are associated with CJD. This disorder is a result from a mutation in the gene that regulates the production of the human protein or direct transmission with abnormal protein in infected brain tissue. Scientist believe that there is a transmissible agent that is responsible for causing CJD. This is a slow virus, since it can take many years from exposure to the appearance of

This disease gets its name from the German neurologist Hans Gerhard Creutzfeldt and Alfons Maria Jakob that first identified this disease. (Victor, 2015) VCJD is primarily found in the brain of the cattle. Abnormal proteins called prions have thought to be the cause of this disease in both cattle and in humans. These prions cause tiny holes that look like a sponge under a microscope. (Melissa Conrad Stöppler, 2015) Unlike most viruses and bacteria these prions unfortunately do not die when exposed to heat, ultraviolet light, and radiation. Disinfectants that are usually used to kill viruses and bacteria also do not work to kill prions. Even cooking the meat very well will not lower the risk of prions in the

While dysfunction of prion proteins remains the most widely accepted etiology of BSE, the USDA suggests there may be two other possible theories that could better explain the manifestation of BSE: the virino theory and the virus theory. However, both theories pale in comparison to the robust evidence in support of the prions theory. A major argument that works against both the virino theory and virus theory is that throughout several studies, the use of various treatments known to damage or inhibit nucleic acids have had no effect on the transmissibility of BSE. Interestingly, prion proteins lack nucleic acids – making them an excellent candidate as the infectious agent responsible for the development of bovine spongiform encephalopathy.

Many scientists hypothesize that protein dysfunction plays a role in the progressive damage and death of nerve cells. The nerve cells may be blocked from communication

It is a rare, degenerative but fatal brain disorder affecting very a small fraction of persons. The symptoms usually arise at the age of 60 and the person dies within a year. Many researchers believe that this disorder is the result of an abnormal protein known as prion. About 5-10% cases reported in the United States share a genetic basis where this form of dementia is caused by a mutation in the gene for the prion protein. Patients with Creutzfeldt-Jakob disease suffer from the problems associated with muscle coordination, personality changes, impaired memory, judgment making, thinking disability and impaired vision. Other possible symptoms include insomnia and depression. In later stages the persons

"Mad cow" disease is a deadly disease caused by prions in the brains of cattle. The real name of this disease is bovine spongiform encephalopathy (BSE). Abnormal prions are usually found in the brain tissue of many diseased cattle and they transmit the infection. Many changes are seen in the brain of infected cattle. Infection can lead to small holes in parts of the brain, giving the tissue a sponge-like appearance when viewed with a microscope. These small holes cause slow decline in a cow’s brain and eventually other symptoms develop affecting the whole body. The cows then die.

BSE is caused by the misfolded prion protein known as PrPsc which originated from PrPc protein. Prion proteins are formed when PrPc becomes a misfolded polypeptide chain – a change in the shape of the protein. PrPsc influences the folding of PrPc multiple times leading to aggregates to form fibers that

Prions cause diseases, but they aren’t viruses or bacteria or fungi or parasites, but proteinaceous infectious particles, an abnormal form of a normally harmless protein found in the brain causing variety of fatal neurological diseases of both animals and humans. They are the ones that cause the well-known “mad cow” disease in Britain and “Scarpie” for animals. Prions act slowly, and are virtually indestructible.

Creutzfeldt - Jakob disease is a prion disease characterized by the degeneration of the cerebral cortex, basal ganglia, cerebellum, brainstem and spinal cord. CJD is classified into 4 types: sporadic, familial, iatrogenic, and contaminated meat. CJD represents approximately 85% of all human prion diseases . Although it is common in older ages (50-70), both men and women have an equal chance of acquiring the disease. Recently, there has been a new variant CJD that is thought to be from the ingestion of cattle products in Great Britain. The central characteristic that differentiates vCJD and CJD is that it occurs more commonly in younger people and also has different pathogenic findings. The second variant form is the panencephalopathic form, which is found in Japan.

Transmissible spongiform encephalopathies (TSEs) are neurodegenerative diseases that are thought to be caused by the misfolding of prion proteins. Prions are able to replicate in the absence of nucleic acids. TSEs include: scrapie, bovine spongiform encephalopathy, Creutzfeldt-Jakob disease, kuru, Gerstmann-Straussler-Scheinker disease, and Fatal Familial Insomnia. They can affect many different animals, including humans. Currently, there are no ways to diagnose, treat, or cure TSEs, as much more research is needed before these diseases are completely understood.

Our current society is surrounded by the debatable usage of vaccines and its importance around the world. Since the introduction of immunizations, many diseases have been decreased and even eradicated from humanity. Even though this is true, serious side effects along with lifelong illnesses follow the temporary cure of viruses. A vaccine is a substance that delivers immunity against viruses and is used to stimulate the construction of antibodies. Vaccines are prepared from the disease itself treated to perform as an antigen without inducing the disease (Vaccines). These inoculations are used worldwide and various unknown infections have been speculated as the originations for them. Vaccines were originated as an alternative to cure viruses among people but the harmful symptoms, unknown links to incurable