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
The Effects of Transmissible Spongiform Encephalopathies on Humans Abstract Humans have to deal with many different diseases and the ones most disliked are the ones with no cures. Like cancer, transmissible spongiform encephalopathies have no cure, but they are more rare. These diseases are prion diseases which cause the brain to deteriorate.
It was a challenge for researchers to find a cure because of how new these strands were, and there was also pressure to find these cures soon due to how fast this virus could have, and in some cases did, move through populations. Shortly after his expedition to Kitum Cave, Gene Johnson says this quote from Louis Pasteur, a 19th century microbiologist, “Chance favors the prepared mind.” This quotation applied to the situation because those who knew how to handle this virus ended up being far more successful than those with no experience. Therefore, the author aims to point out that those who treated the virus with ignorance had created danger by approaching their situations with carelessness, which ultimately leads to
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).
Concluding that this virus had mutated to become a different strain, scientists also understood that the pathogens adapted to the immune system and were immune to its defenses so
Creutzfeldt-Jakob Disease: It happens when a prion protein misfolds itself, thus causing a “domino effect” which unfolds itself causing a malfunction.
Scientists are sharpening their swords once again preparing for battle. This vehemence was in sued by new evidence favoring the underdogs of the last campaign, which took place in “1946” (dis. Rice). That hatchet has been buried for 70 years, only to be excavated and used in a global game of hot-potato. The cause of this contention and angst is a microscopic composition of proteins and enzymes. Though it may not seem like a controversial subject, highly intelligent minds are on both sides of the debate. They argue over the question “Is a virus a living or non-living?”
their host. Pathogens can be microscopic or macroscopic. Prions are Infectious protein that causes the degeneration of brain tissue in mammals. A form of proteins that have undergone alteration form
Bovine spongiform encephalopathy is the scientific name for the disease commonly referred to as mad cow disease (MCD). (WebMD, LLC, 2015) MCD gets its name because when a cow has this disease it stumbles around and even gets demented or mad. (Chris Woolston, 2015) This disease effects the central nervous system,
B) Similar disease in human C) This essay will discuss possible causes, effects, and treatment for this disease. II] Causes Transmission from animal to human. Transmission from human to human. Genetic mutations in animal and human. III] Effects Cow Human IV] Treatment and control No treatment available Isolation and destroy affected animals. V] Conclusion A) BSE is a slow fatal neurodegenerative disease affecting adult 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
J. Thompson (2014) 2. In a healthy cell floating in the cytoplasm are proteins we refer to as “chaperones” because they instruct or guide the folding of younger proteins. They guide them through the folding process resulting in perfectly structured protein. That performs its function as it should. When chaperones go bad aka Prions, as is the case in Bovine Spongiform Encephalopathy (BSE), we see the wide spread destruction that can occur when mutations in the folding occur. In Bovine Spongiform Encephalopathy the PrPsc protein acts as a chaperone and influences the PrPc protein it comes in contact with, to misfold which results in a new structure this new form iis hydrophobic (PrPsc). As these proteins form they aggregate with other PrPsc proteins. As the PrPsc proteins aggregate they trigger the neuron to self destruct which
However, differing from the other diseases, Prion sometimes spreads through contaminated meat. In the article “Prion Diseases” it shows meats that have mad cow disease can cause prion disease. Intake of unhealthy meat is not infectious since the spread is not direct. Moreover, like Alzheimer’s, transferals through medical procedures may cause the gain of Prion Disease. Prion disease can be the result of a transfusion with a person with the disease. Furthermore, many things may take a toll on the spread of Prion disease, but it is not
Prion Diseases Abstract 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.
completely proved. Prion stands for “proteinaceous infectious particles”. Prions are known to cause many diseases involved with nervous systems like the brain. They are the ones that cause the well known “ mad cow ” disesase in Britain and “scrapie”