Prions: Proteinaceous Infectious Particles
A Prion is a controversial infectious agent made up of misfolded proteins, which, unlike all other known infectious agents, doesn’t contain any nucleic acids (KJ Ryan 624). Prion molecules are the cause of numerous fatal diseases which attack sensitive neural tissue and are, as to date, virtually untreatable mostly due to inadequate awareness and funding for research (Prusiner). A well-funded focus on Prion research could bring us closer to finding cures for the associated diseases as well as unlock doors to more information on neural infections in general.
Prions were discovered by Dr. Stanley Prusiner in 1982 after one of his patients died of Creutzfeldt-Jakob Disease. He was able to trace the cause
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Another possible treatment is the use of antibodies. A research team was able to completely eradicate prions from infected cells in a dish, but the trials done on mice only produced results under certain conditions. The MRC unit in London has discussed the development of an anti-prion antibody, but no plans for testing in the near future have been announced.
Administering antibodies for a neurological disease faces another one of the many walls blocking researchers from a cure. Being as Prions attack the brain, most hypothetical methods present a risk of damaging the neural tissue even further. Any medical treatment to the brain requires crossing the Blood-Brain Barrier, a tight clump of cells protecting the brain from flowing blood. However, the MRC has found evidence that suggest the Blood-Brain Barrier may be able to be broken down in Prion patients.
The most recently proposed solution is the hypothetical and highly experimental “Gene Silencing” treatment, which suggests the use of DNA or RNA to temporarily turn of a specific gene. Theoretically, if the Prion Protein Gene were to be turned off the body would stop producing the Prions and effectively curing the patient, however it is very far away from becoming a reality in human patients
Quanah Parker was born in 1845, the exact date of his birth is not known due to the times and the lack of recording dates like birthdays back then. Also the exact place of his birth is unknown, it is thought to be somewhere along the Texas-Oklahoma border, but there are conflicting reports. Quanah himself said that he was born on Elk Creek south of the Wichita Mountains, but a marker by Cedar Lake in Gaines County, Texas says otherwise. There are still other places where he was supposedly born like Wichita Falls, Texas. “Though the date of his birth is recorded variously at 1845 and 1852, there is no mystery regarding his parentage. His mother was the celebrated captive of a Comanche raid on Parker's Fort (1836) and convert to the Indian way of life. His father
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...
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...
PrP can occur in two forms- a normal cellular prion protein known as PrPc and a pathogenic misfolded conformer known as PrPsc. The abnormal PrPsc differs from the normal prion protein PrPc in both secondary and tertiary structure. PrPsc is principally rich in Beta sheet contents but PrPc is principally rich in alpha helical contents. Due to this difference of between the isoforms, prions are extremely resistant to certain decontamination systems. The Two tables below outline both human and animal diseases (2).
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.
The prion diseases that Chronic Wasting Disease is related to are Creutzfeldt-Jakobs disease found in humans, bovine spongiform encephalopathy (BSE) in cattle, and scrapies in sheep (3,4). These diseases are grouped together because they share certain characteristics such as long incubation periods, spongiform changes that are associated with neural loss, and cause failure to induce inflammatory responses (Chronic Wasting Disease Alliance).
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.
Gene therapy gives people who suffer from genetic diseases a chance to lead a normal life. Dangerous diseases, such as AIDS, SCID, Thalassemia and ADA can be cured successfully. In September 5, 2006, two people with advanced melanoma received Gene therapy and they got recovery soon. This is a breakthrough in cancer gene therapy. Gene therapy uses patients own cells to cure diseases, and, therefore, no rejection to their bodies. Furthermore, patients could get permanent cure from gene therapy without recurrence.
...t one has it. Many scientists hypothesize that there is an inflammatory response in the brain when there is an extensive B-42 amassing. To slow or stop this, scientists theorize that the use of anti-inflammatory drugs, such as aspirin, could delay the swelling in the brain. Also, as scientists have known that taking a multi-vitamin tablet each day is good for you, there has been recent research demonstrating that the use of antioxidants may protect neurons, not just the immune system and keeping the body healthy by providing vitamins and minerals. These special supplements protect the neurons from the effects of the accruing B-Amyloid that would likely cause the plaque that causes AD.
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.
Prion proteins are encoded by the Prnp gene, derived from the Prn gene family. This gene codes for a 254 amino acid protein, which, during posttranslational modification, is truncated to its wildtype 209 residue cellular prion protein (PrPC) form.1 PrPSc is the pathogenic form of PrPC, which primarily differs in secondary and tertiary structure. A protease resistant, 142 amino-acid pathogenic form, called PrP 27-30, is also sometimes derived from the cleaving of PrPSc.1 After posttranslational modifications, PrPC is then anchored extracellularly to the cell surface by its C-terminus to a glycosylphosphatidylinositol (GPI) anchor in a lipid raft.1 It also has two N-linked oligosaccharide chains. 1 Additionally, the protein can be found
Interferons were first discovered as a result of their ability to prevent viral replication, by Alick Issacs. After some experimenting with heat damaged flu virus he notice something interfered with the replication of the virus in cells. He called this substance Interferons. Since them additional research has determined that interferons also affect tumor growth and can be used to fight certain types of cancers.
Fatal neurodegenerative diseases, such as kuru in humans and bovine spongiform encephalopathy in cattle (commonly known as “mad cow disease”), were shown to be transmitted by prions. This is very interesting because not everyone knows this and believe that everything is just spread through viruses. That is not the case, there are different way that different diseases get spread. The disease commonly known as (“mad cow disease”) is spread by the consumption of meat, nervous tissue, or internal organs between members of the same species. You cannot destroy prions by
Genes are made of DNA – the code of life (Gene Therapy- The Great Debate!). The changes in genes may cause serious problems, which we called genetic disorder. In theory, the only method to cure genetic disorders is gene therapy, which basically means the replacement of genes in order to correct the loss or change in people’s DNA. Although gene therapy gives patients with genetic disorders a permanent cure, it is controversial because it has safety and efficacy problems, and raises ethical issues.
The procedures that will be the future of modern medicine currently fall into the realms of taboo and fictional. These procedures encompass every aspect of medical science, from exploration of the human body, curing diseases, to improving a person’s quality of life. Many of these procedures are not very well known, while a few have been in the spotlight. These procedures include cloning, nano-robotics, retro-viruses, and genetic manipulation via gene-specific medications. For any serious breakthroughs in modern medical science, we must embrace these new forms of treatment instead of shying away from them. Second, I’ll attempt to explain how these methods and procedures could benefit mankind.