Prions Disease Summary- Alex Schray
Causes:
Prion diseases are caused by proteinaceous infectious particles (more commonly referred to as prions). According to the CDC the term prion “refers to abnormal, pathogenic agents that are transmissible and are able to induce abnormal folding of specific normal cellular proteins.” Prions are always contracted one of three different ways. They can develop sporadically through a sudden genetic mutation. They can be inherited; prions are autosmol dominant, meaning that children of the infected have a fifty percent chance of inheriting the disease. Finally, prions can be ingested: either through consumption of infected meat or after exposure to poorly cleaned medical instruments.
Symptoms: Creutzfeldt-Jakob
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In prions, however, the PRP primary structure remains the same but the secondary structure does not. The alpha helices which typically comprise most of the secondary structure are replaced by beta pleated sheets. This structural mutation makes prions especially dangerous. The increased number of beta sheets means prions cannot to be broken down by protease and thus have the un-prohibited ability to convert other proteins to prions. This mass conversion eventually causes holes to appear in the brain as grey matter deteriorates. This degeneration of the brain results in extreme changes in the nervous and muscular systems. As the brain develops holes from the prions, all function controlled by the brain begins to malfunction. Movement for example, controlled by and large by the cerebellum, becomes extremely difficult and disjointed when one has CJD- because of the deterioration of the grey matter of the cerebellum. The hippocampus, responsible for memories, loses its hippocampal grey matter as prions convert PRP molecules, causing the largescale loss of
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).
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. Prions are proteins that sometimes behave like viruses, which mean that they should have some form of nucleic acid, but since they don’t, they cause abnormalities. The nervous system contains many normal prions, but when an abnormal prion comes along, it transforms all the normal prions into abnormal ones. Bovine spongiform encephalopathy is found in cattle, but it can be transmitted to humans.
Bradbury uses Montag’s view of Mildred’s characterization to convey that Mildred exemplifies a bad character with her obsession with technology and consequential treatment with Montag. Mildred’s decision to prioritize her self desire for technology damages her relationship with her husband, Montag. Montag meets with an english professor named Faber who also believes that books need to be preserved. They have a huge discussion about how they should get everyone aware about the books. Montag is frustrated because “nobody listens any more.
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
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
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
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
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
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.
Since the Rockefeller Drug Laws were passed in 1973 under Governor Nelson Rockefeller, New York State has had the harshest sentencing for low-level, non-violent drug offenders of any other state in the nation. Under these laws, those convicted of drug offenses face the same penalties as those convicted of murder, and harsher penalties that those convicted of rape. (Sullum, 1) Though the laws were first enacted to curb the late-1960s-early-1970s psychedelic drug epidemic, New York's drug problem in fact worsened in the 1980s with the use of stimulants, and thus the laws were reformed to be less lenient. These unforgiving laws, which place enormous minimum sentences for drug-sale convictions, prove to be ineffective and expensive and have
Prions are a type of protein found naturally in the brain and other regions of the central nervous system. The diseases associated with
The French Revolution was set off by income inequality by French citizens. “Income inequality was only a by-product of a much more complex problem, a class of nobility with utter disdain for the peasants and their sufferings” (Adams). The peasants and the low class were minor to the nobility and it was held by church laws. Almost all the problems that occurred in France were blamed on the low class. After the low class got taxed a lot it led to hungry civilians who caused an uprising against
Imagine deteriorating from the inside out. Imagine losing control of everything. Every. Single. Thing. Imagine suffering for an entire lifetime. Creutzfeldt-Jakob disease (CJD) is a human prion disease caused by a normal prion protein that becomes misshapen into a harmful prion that builds up in the brain and disrupts normal brain function. It is a rare disorder that is fatal, usually within 6 months of diagnosis and a year of the first symptom. In the United States, there are about 300 new cases per year. Creutzfeldt-Jakob disease shuts down the brain and eventually causes death.
Chronic Wasting Disease (CWD) is a fatal neurodegenerative transmissible spongiform encephalopathy (TSE ) found in some white tailed deer. CWD is associated 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.
As a medical practitioner, I was always fascinated by the complexities of the human nervous system. A few years later, during my neurology residency training, I developed special interests in the field of neurodegenerative disorders and dementias. These are largely non-curable disorders