History of Prions

People of the Old World had domesticated pigs, horses, sheep, and cattle(1), which had acted as pathogens to infect the Europeans with diseases. In addition, diseases were constantly circulated with centuries of war, exploration, and city building. During the process of natural selection, disease-intolerant

In the early 1900’s, the Fore tribe was struck with an epidemic of Kuru, a brain eating disorder that causes shaking, and furthermore the name “Kuru” in the means shaking or trembling in a literal sense. The Fore spread this disease a fair bit, specifically due to their cannibalistic funeral practices of eating the remains of the dead, and specifically the brain (Bichell, Rae Ellen 2016). However, the presence of Prions have been around for eras, but the discovery in the Fore tribe spearheaded the movement to learn more about them. Eventually in 1982, the “Prion protein” was discovered by Stanley Prusiner, eventually earning him the Nobel Prize in 1997 for his proof of the lack of DNA or RNA in the protein, juxtaposed to bacteria and viruses (Prion Alliance, 2016). As the amount of diseases discovered increased, it became eerily known that the Prion disease in humans and animals were all caused by one original misfolded protein, PrPsc. Despite a common origin, pinning down a solution to the problems of Prions is an extremely complex issue. With an extreme resistance to everything, Prions opens the question of sanitization in hospitals. If a doctor were to be working with a Prion-contaminated sample, the tools used may be thought to be completely clean, yet Prions being highly resistant would persevere against normal

Individual who were having illusions or were delusional had a hole drilled in their skull in order to get rid of the spirits. If that person was still alive, the procedure was successful

infection not only hit the humans but animals were also able to contract it. Not many people

suffered from was called Encephalitis Lethargica (Saxon). A doctor was called in to treat the

Humans are susceptible to many different diseases and unfortunately, these transmissible spongiform encephalopathies have death written all over them.

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.

During the early to mid 20th century, not much was known about mental illnesses or what caused them, which was the way it had been for many years before. One of the somewhat common perceptions about mental illness was that these disorders were caused by possession or negative spirits. This can be most likely be attributed to the wide influence of the catholic church and the belief that exorcisms or psychosurgery could possibly cure incurable or intractable mental illnesses. Psychosurgery developed into the more common procedure known as a lobotomy, where the connections between the prefrontal lobe and the prefrontal cortex are cut with the intention of freeing the patient from delusions and side effects of other mental illnesses. Lobotomies began as a surgical procedure which needed to be performed in an operating room, as it required holes to be drilled through the scalp and into the skull. This meant that though the procedure was seen to show some

The U.S. Department of Agriculture (USDA) has tested hundreds of thousands of cattle for BSE. Researchers 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 cattle (http://www.cdc.gov). According to The National Creutzfeldt-Jakob Disease Surveillance Unit, by June 2014 it had killed 177 people in the United Kingdom, and 52 elsewhere. This essay will focus on the possible causes, effects, and treatment for this

The aggregation of prion proteins and their transmissibility from one cell to another has been shown to be evident (Cushman et al.; Goedert et al. 2010), therefore strongly suggesting that these events may play a role in pathogenesis for many diseases, including both AD and PD. While none of these diseases is infectious in an identical way as

In addition to the cervid species in which chronic waste disease can occur in wide variety of other animals. These other animals include monkeys, sheep, cattle, prairie voles, mice, and ferrets. Environmental transmission has been linked to contact with infected bodily fluids and tissues. Once the prions are in the environment chronic waste disease prions may remain infectious for many years. Thus, Decomposition of the disease carcasses, infected gut piles left by hunter, as well as urine, saliva, feces, and antler velvet of infected individuals that are deposited in the environment.

The first known case of Exploding Head Syndrome was described by Silas Weir Mitchell, an American doctor. He published a paper called “On Some of the Disorders of Sleep” in the Virginia Medical Monthly in 1876. He describes a patient identified as “Mr. V” and his experiences of noises that sounded like a gun being triggered, a bell being sounded, and a guitar being broken with a twang-like noise. A Welsh psychiatrist named Sir Robert Armstrong-Jones described many of his patients experiencing nightmares that they described as a “snapping of the brain” in 1920. A neurologist named John M.S Pearce published an article in the 1988 journal of Lancet called “Exploding Head Syndrome”. In the article he explains the symptoms of 10 patients who all

Prions are a type of protein found naturally in the brain and other regions of the central nervous system. The diseases associated with

In 1959, the first ever human prognosis of a prion disease was found and it was named Kuru, which was mainly caused by ritualistic cannibalism. In history, Kuru was one of the main causes of death for native people, especially Fure women. Between the 1920 and the 1960 an epidemic of Kuru swept over the Fure people; killing around 200 people a year. This disease killed many women and children because they ate the brains of their deceased. In contrast, the men were less affected by the disease because the majority of them only feasted on the muscles. A supplementary of the native people who had Kuru showed symptoms such as trouble walking and cerebellar dysfunctions. Gajdusek had described the Kuru disease and his description led Hadlow, a researcher,