How to Treat Prion Diseases

1635 Words4 Pages
bulb-icon

Recommended: Essay on prion disease

How to Treat Prion Diseases

Abstract

Scientists are stumped as to the development and nature of proteinaceous infectious particles. Neither virus nor bacteria, these prions, are believed to cause transmissible spongiform encephalopathies (TSE), rare diseases said to be 100% fatal, without possessing nucleic acids. Their unhindered growth is thought to be the cause for bovine spongiform encephalopathy (BSE), or Mad Cow Disease, Creutzfeldt-Jakob (CJD), scrapie and other TSE, diseases characterized by the brain microscopically turning into sponge-like matter. There are no cures or effective treatments available today because drawbacks constantly prevent the development of efficient therapy. Studies continue to slowly progress, hoping to find methods to immunize against more prion diseases.

Problem

No one presently has a solid understanding as to why TSE, or prion diseases, occur. The simple explanation is that PrP(c), the normal isoform of the prion protein, is forced to fold into PrPSc, the other pathological isoform, causing the misfolded PrP(c) to acquire protease-resistance. As to a physical presentation, a clumped protein consisting primarily of alpha-helices (spirals) is converted into one consisting primarily of beta-sheets (sets of pleated hairpins). In an essence, alpha-helical content decreases while beta-sheet content increases. The newly converted protein then possesses the same characteristics as those of the native PrPSc (Korth, Streit, & Oesch, 1999).

PrPSc acquires partial protease resistance upon the transformation and passes this resistance along to the naturally protease-sensitive PrP(c). Protease is an enzyme that breaks down proteins or peptides, deeming the protease-sensitive proteins soluble; so during ...

... middle of paper ...

...r to the fatal TSE.

8

b10

References

Bainbridge, J., Jones, N., & Walker, B. (2004, May 12). Multiple antigenic peptides facilitate generation of anti-prion antibodies. Retrieved July 26, 2008, from http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=1809119#

Graham, S. (2002, July 30). Common Antibiotic Saps Prions' Strength. Retrieved July 24, 2008, from http://www.sciam.com/article.cfm?id=common-antibiotic-saps-pr

Korth, C., Streit, P., & Oesch, B. (1999). Monoclonal Antibodies Specific for the Native, Disease-Associated Isoform of the Prion Protein. Methods in ENZYMOLOGY: , 309, 106-122 .

Soto, C. (2006). Prions: The New Biology of Proteins. Taylor & Francis Group: CRC Press.

Wong, K. (2001, August 14). Old Drugs Show New Promise in Combating Prion Diseases. Retrieved July 24, 2008, from http://www.sciam.com/article.cfm?id=old-drugs-show-new-promis

Show More
Related

  • Understanding Pharmacology: Effects and Interactions

    726 Words  | 2 Pages

    Pharmaceuticals have examined and found to ”work by changing the biological functions of the target cells in the body through chemical agents“ (Doweiko, 2015, p. 16). ”Many people in the past have thought that drugs that

    Read More

  • Bovine Spongiform Encephalophathy

    976 Words  | 2 Pages

    Guyer, Ruth Levy, Ph.D. “Prions: Puzzling Infectious Proteins” National Institutes of Health Office of Science. 28 July 2006 < science.education.nih.gov/nihHTML/ose/snapshots/multimedia/ritn/prions/prions1.html>.

    Read More

  • The Horrifying Details of Mad Cow Disease

    2561 Words  | 6 Pages

    Mad Cow Disease, scientifically referred to as (BSE) Bovine Spongiform Encephalopathy, is a disease that affects those humans who eat the meat from infected cows. Mad Cow Disease is one of several fatal brain diseases called (TSE) Transmissible Spongiform Encephalopathy. (USDA) There was evidence of a new illness resembling the sheep disease scrapie. It was technically named BSE but quickly acquired the mad cow tag because of the way infected cattle behave. (CNN) In 1997, there was an award given to Stanley Prusiner, for concluding that a distorted protein called a prion was responsible for Mad Cow Disease, noted the long incubation period made it difficult to distinguish (Bryant). Another name for Mad Cow Disease is the new variant Cruetzfeldt-Jakob Disease (vCJD), similar to the Creutzfeldt-Jakob Disease, which is a deadly brain illness that strikes about one per million per year (USDA) due to genetic or unknown causes while the vCJD is contracted from eating infected cows (USDA). Both CJD and vCJD are so similarly named because of the similar effects from the illness.

    Read More

  • Mad Cow Disease: Eradicating A Cow Killer

    517 Words  | 2 Pages

    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...

    Read More

  • Creutzfeldt-Jakob Disease Research Paper

    535 Words  | 2 Pages

    Creutzfeldt-Jakob is known as a prion disease. Prion is a protein that occurs normally inside the brain, however

    Read More

  • Chronic Wasting Disease (CWD)

    1090 Words  | 3 Pages

    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...

    Read More

  • Prion Disease Essay

    2380 Words  | 5 Pages

    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).

    Read More

  • Mad Cow Disease

    854 Words  | 2 Pages

    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.

    Read More

  • Chronic Wasting Disease

    1491 Words  | 3 Pages

    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).

    Read More

  • Bacteria, Viruses, and Prions

    758 Words  | 2 Pages

    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.

    Read More

  • Prion Disease Essay

    2219 Words  | 5 Pages

    Prion Disease is an illness that many have not heard about. This is sad because many have died and are dying from this disease that doesn’t yet have a cure. “Prion Disease is a group of conditions that affect the nervous system in humans and animals… these conditions impair brain functions, causing changes in memory, personality, and behavior; a decline in intellectual function (dementia); and abnormal movements particularly difficulty with coordinating movements (ataxia)” (Genetics Home Reference). This is basically the definition of what Prion Disease is and without going into depth it explains how it affects the person that is affected. “In t...

    Read More

  • HEPATITIS C

    1006 Words  | 3 Pages

    “This knowledge will help us design drugs that mimic the viral effects on these proteins to either activate a host’s immune response or shut it down,” said Dr. Michael Gale, associate ...

    Read More

  • Creutzfeldt-Jakob Disease Essay

    960 Words  | 2 Pages

    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.

    Read More

  • The Evolution of Antibiotic Resistant Bacteria

    1427 Words  | 3 Pages

    Bibliography:.. References 1) Lewis, Ricki, “The Rise of Antibiotic-Resistant Infections”. Food and Drug Administration Publications. http://www.fda.gov/fdac/features/795_antibio.html September, 1995. 2) Levy, S., Bittner, M., and Salyers, A. Ask the Experts about “Ask the Experts”.

    Read More

  • Is Suicide the Solution?

    1534 Words  | 4 Pages

    Ed. David Zieve. U.S. National Library of Medicine, 26 Feb. 2014. Web. The Web.

    Read More

More about How to Treat Prion Diseases

Open Document