Out of the many forms of infectious agents, one that seemingly defies the central dogma of biology is the prion. These types of diseases are created when an otherwise suitable protein is altered in a prion like domain, becomes misfolded, and begins contorting other specimens of the same protein to this misfolded shape resulting in what’s known as an amyloid fiber. This entire replication process does not require DNA, simply more of the non-mutated protein. Recent studies focusing on mutations identified in two heterogeneous nuclear ribonucleicprotiens, hnRNPA2B1 and hnRNPA1, have shown to produce cytoplasmic inclusions. This causes mass degradation of muscle tissues and structures throughout the human body known as multisystem …show more content…
Results of exploration Creation and Spreading of Prions
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
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).
There is now strong scientific suspicion that the agent responsible for the outbreak of prion disease in cows, bovine spongiform encephalopathy (BSE or 'mad cow' disease), is the same agent responsible for the outbreak of vCJD in humans. Variant CJD (vCJD) is not the same disease as classic CJD It has different clinical and pathologic characteristics. Each disease also has a particular genetic profile of the prion protein gene. Both disorders are fatal brain diseases with unusually long periods measured in years, and are caused by a transmissible agent called a
This paper explores the practice of the APRNs autonomy in the state of Georgia and compares it with other states. Also to advocate for policy recommendations of the APRNs scope of practice that are needed to improve the healthcare in the state of Georgia. APRNs are registered nurses that provide continuous care and treatment in many different areas, such as pediatrics, primary care, acute care, maternity, mental health and chronic disease management. APRNs also have advanced education, training, and national certification in specific areas of practice. Even with the changing and expanding of healthcare, APRNs continue to face challenges in practicing to the full extent of
Prions (PrPsc) influence the PrPc proteins they come in contact with to misfold and begin to aggregate. The aggregation leads to development of fibers or plaques. The aggregation of PrPsc around the neurons triggers the cell to self destruct. It is also able to spread to nearby neurons resulting in areas of neuronal death which results in the brain tissue having spaces of void which appear like a sponge.
Nelson is perched on the shores of Kootenay Lake and offers a wide-range of world class recreational activities. The stunning geography and outdoor activities, however, are only part of Nelson’s overall charm. The community still holds true to its silver mining roots as is evident with its impressive selection of over 350 restored heritage buildings and many of these unique buildings make up the bustling downtown core of the city.
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
Question ONE (to be answered as a straight-forward philosophical essay): Silenus, the satyr, is, or perhaps pretends to be, a determinist. After all, everything about the satyr, both his nature and nurture, come directly from his creator, Hench. And certainly, at the beginning of the story, Hench accepts full responsibility for the satyr’s actions. But he eventually changes his mind. How can the satyr, all of a sudden, become morally responsible for his actions? What exactly changed in Hench’s thinking? Michael Gorr is a compatibilist. Does his position make better sense than either the determinist or the libertarian? Explain.
Daniel Solove writes in his article that the “nothing to hide” argument is a terrible argument, because privacy is important to everyone and everyone has something to hide. Solove writes that if you break the argument down to its core the argument means nothing. Solove addresses the argument for the “nothing to hide” belief by stating why some agree with the argument because the argument can be protection against acts of terror, then describing how the government knowing everything on someone can cause plenty of problems as he describes. Solove takes the explanation of privacy to a place that most people ,if not all ,can relate to just to define privacy more than the general consensus. Solove gave a few
Netherlands’s companies and industries should understand and study the political-legal environment since it is a critical concern of a successful global organization. As stated, Netherlands is a developing country so its government is one of those that exercise control of the businesses and trading by implementing laws and regulations (e.g. tax breaks and tariffs privileges). This could affect the products to be exported in other country/ies. To have a better and fair competition, government established antitrust laws and regulations. One of the factors that could affect the country’s industry, company and trading products are the political risks. These risks could affect the company’s capacity to do productively and profitably. This will lead
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.
I chose to write about the short story "A&P." The story takes place in a small town in the late 60's, in a vacation town of sorts with a general store, few residents. What I received from the setting, was a very low maintenance town where "everybody knows everybody" (very tight community) most likely old school beliefs and structures (religion, dress code, ethics, morals, ext strict) and for a short time tourists come to live for a while, and in a sense shake up the foundation of the town a bit with their outside beliefs and values.
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
CJD is a serious neurodegenerative disorder, a rare form of rapidly progressive dementia, with a 100% fatality within one to two years of onset. CJD belongs to a family of human and animal diseases known as the transmissible spongiform encephalopathies (TSEs). It is believed to be caused by an abnormal isoform of a cellular glycoprotein known as the prion protein that becomes toxic in an abnormal form (CDC) although Dr. Campellone suggest other proteins may play a role in this disease as well. Although, it is so rare that only between one to every one million people worldwide are diagnosed.
Now prions work in an odd way, prions replicate in conformation and are a form of peptide bond (The Dangers of Cannibalism: Ellington, Andy). Then, a
to the amino acid, and it is half the size of hemoglobin. PrP is the protein,