In the study of micro-biology, there are several kinds of pathogens that can be observed in the human body. They individually can pose a risk ranging from totally harmless, to life threatening, depending on the pathogen. There are types of bacteria, virus, and even fungus that can cause a wide range of problems for the human body. But there is one type of pathogen that is just as elusive as it is dangerous – a pathogen that is rarely discovered in the human body, yet when it is, doctors are at a loss as to what can be done to stop it. It is called a Prion.
A prion is a protein with a three-dimensional shape, as opposed to standard proteins. When a prion comes into contact with another protein, that protein transforms into another prion. Eventually, as more proteins come into contact with prions, the host will show symptoms of a severe and ultimately fatal illness. Common diseases caused by prions would be Bovine Spongiform Encephalopathy (known informally as “Mad Cow disease”), and Crutzfeldt-Jakob disease. There are also strains of diseases infecting other mammals as well, including Elk (Chronic Wasting disease) and Mink (Transmissible Mink Encephalopathy). One common connection is that all prion-caused diseases primarily damage the brain tissue of the patient. All known prion diseases are fatal, and have no cure or treatment.
Prions were first brought to the attention of the medical community by a man named Stanley Prusiner. Prusiner brought up the concept of prions with the theory that there was an infectious protein within animals and humans, along with the standard proteins that are constantly passed through our numerous cells. The idea of a protein capable of infecting other proteins didn't sit well with his colleagues, but...
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...covered. If it is true that prions don't cause BSE or CJD, then what does? If they do, what can stop a prion – or can they be stopped? A prion is, after all, a type of pathogen that was only recently identified.
There is a lot that we still do not understand about prions. All cases of prion-caused diseases are terminal, and doctors are entirely helpless. Perhaps, in the future, a method of combating this pathogen will be discovered. Until that time comes though, prions remain essentially invulnerable to us. The only measures we can take in protecting ourselves from diseases caused by prions are purely preventative. Stricter food production monitoring, making sure no brain matter is distributed to the populace, and other ways to halt the spread of diseases. And yet, sometimes people just get CJD sporadically. Prion caused diseases are, essentially, a medical mystery.
Mad cow disease is caused by prions, "weird mutant proteins that are found in brain and spinal tissue"1. Another name for mad cow disease is called bovine spongiform encephalopathy (BSE) and the definition is "a progressive neurological disorder of cattle that results from infection by an unusual transmissible agent called a prion"2. It started from what is called a prion protein then it turned into a pathogenic, and then it starts to damaged the brain of a cattle. There's another name for this disease and it's called Creutzfeldt-Jakob Disease "a form of progressive dementia characterized by loss of nerve cells and degeneration of nerve cell membranes leading to the production of small holes in the brain. It is rare, degenerative, and invariably fatal"3. This disease happen in human causing lapses in the memory, mood swings similar to depression, lack of interest and social withdrawal3. It is said that this disease has no tr...
Creutzfeldt-Jakob is known as a prion disease. Prion is a protein that occurs normally inside the brain, however
In sporadic CJD, the disease occurs even though the affected does not have any known risk factors that would cause an occurrence of the disease. This sudden occurring CJD is indisputably the most frequently diagnosed type of Creutzfeldt - Jakob disease. This statistically accounts for at least 85 percent of CJD cases. Due to that there are some fifty to sixty deaths per year due to sporadic CJD in the United States alone. Similar figures are seen in other countries such as Australia, Canada and the United Kingdom.
The origin of CWD has yet to be determined (Sigurdson & Aguzzi, 2007). The infection was first noted in 1967 at a captive mule deer research facility. In 1978 pathologists recognized the TSE type brain lesions, also that CWD presented as a prion disease by the neuronal perikaryonic vacuoles, the accumulation of aggregated prion protein and prion infectivity in the brain. In the late 1970s and early 1980s the infection w...
TSEs or more commonly prion diseases are a group of invariably fatal neurodegenerative diseases that occur in humans and animals . This disease is caused by a protease –resistant protein (PrPsc) after misfolding of a host-encoded prion protein (PrP). TSEs can exist as genetic, infectious or sporadic forms. The diseases are characterized by dementia, ataxia and neuropathlogically due to loss of specific neurons in the brain. Other clinical features include persistent painful stimuli, dystonia, visual or cerebellar problems and gliosis (1).
Autopsies of affected cattle reveal holes in the brain tissue that give it a spongy, or spongiform, texture. Similar spongiform diseases have been recognized in humans (for example, Creutzfeldt-Jakob disease or CJD) for over a century and in sheep (scrapie) for over 200 years. The cause of BSE is unproven, although there is strong evidence that prions, which may be infective proteins, are the agent. Other hypotheses suggest that prions work with an as yet undetected virus to cause the infection.
Chronic Wasting Disease is a highly transmissible, deadly neurodegenerative disease that affects cervids in North America (Belay et al., 2004; Saunders et al., 2012). There are only four types of cervid that are known to get this disease which include elk, mule deer, white-tailed deer, and moose (Chronic Wasting Disease Alliance). It has been classified has a transmissible spongiform encephalopathy (TSE), otherwise known as a prion disease (Belay et al., 2004). A prion is an irregular, pathogenic agent that causes abnormal folding of specific proteins called prion proteins. These proteins are mostly located in the brain (Chronic Wasting Disease Alliance). The abnormal folding of this protein causes neurodegenerative diseases in a variety of species including humans, sheep, cattle, and deer (Abrams et al., 2011).
Prions are pathogens, and cause infections, like viruses. Prions cause many neurodegenerative diseases, but are made up of harmless proteins found in mammals and birds. The proteins are not in their normal form though, and once they enter the human brain, can cause severe brain infections. One thing that makes them different from viruses, is the lack of nucleic acids, which means they have no genetic code. Once in the brain, they make normal proteins turn into abnormal ones, which then multiply, causing severe infection. Soon, holes appear in the brain that can only be treated by incineration. An example of a disease caused by a prion would be the Mad Cow Disease, or the human equivalent Creutzfeldt–Jakob disease. Prions are very dangerous. While some people can confuse prions and viruses, there are some ways to tell the difference.
Polio is a viral disease. It cripples thousands of people and infects even more every year. Even though millions are inoculated, and the polio disease has been successfully purged from hundreds of countries still thousands of people and developing countries are infected and still people are dying. According to the World Health Organization (WHO) polio affects the Central Nervous System, or CNS; by infesting the intestines and transmitting it into the nerves thought the blood vessels. There the virus spreads through the nerve cells to the brain stem or other motor units, while forever damaging the nerves.
Tamparo, C. D. & Lewis, M. A. (2011). Diseases of the human body. Philadelphia, PA: F.A. Davis Company.
Even though the construction of the BU Biolab was completed in 2008, no research is being conducted since the legal approval from the local community is required for the BU Biolab to fully operate at the BSL-4 research. One of the reasons that keeps local residents from approving is the concern regarding security and safety. As the study of dangerous infections with unknown cure will be taking place at the BU Biolab, the fear of deadly pathogens leaking rises among the public. The ...
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Creutzfeldt-Jakob Disease is an uncommon, deteriorating, consistently fatal brain disorder that is caused by prions. The symptoms of CJD are similar of Alzheimer’s but progress much faster. There are three variations of CJD, sporadic, familial, and acquired. All variations affect the brain the same way and have the same result of death. CJD is an untreatable and incurable disease.
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