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Lynn Nguyen

on 18 April 2013

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Transcript of Prion

Finally Bibliography Prion: Bovine Spongiform Encephalopathy Prion BSE (bovine spongiform encephalopathy) is a progressive neurological disorder of cattle that results from a prion. Definition: an infectious agent composed of protein in a misfolded form. Other Facts... What is it function? The prion exists in two form. The normal (PrPc) can change shape into a harmful disease (PrPSc). It proceeds a change reaction, and can damage neuronal tissue, when PrPSc form long filamentous aggragates. Also known as mad cow disease. by Lynn & Jr All known prion diseases affect the structure of the brain or other neural tissue, and all are currently untreatable and are always fatal. http://www.news-medical.net/health/What-is-a-Prion.aspx http://www.triroc.com/sunnen/topics/prion.htm effects on human: Molecular models of the structure of: PrP^c Prp^sc Predominately a-helix BSE belongs to a family of diseases known as the transmissible spongiform encephalopathies (TSEs). TSE animal diseases found in the United States include scrapie in sheep and goats, chronic wasting disease in deer and elk, transmissible spongiform encephalopathy in mink, feline spongiform encephalopathy in cats, and in humans: kuru, both classic and variant Creutzfeldt-Jakob disease, Gerstmann-Straussler-Scheinker syndrome, and fatal familial insomnia. B sheet (40%) a-helix (30%) http://pdb.org/pdb/101/motm.do?momID=101 http://www.clevelandleader.com/node/18385 http://www.mindfully.org/Health/Mad-Cow-Proteins.htm Diseased prions induce healthy prion proteins to change their shape, and clusters of disease build, leaving holes in the brain. Scientists believe another, separate protein is involved in the conversion of normal proteins into abnormal form.

Source: Dr. Fred Cohen, UCSF http://www.cdc.gov/ncidod/dvrd/prions/ http://brain.oxfordjournals.org/content/128/9/2026.full http://brain.oxfordjournals.org/content/132/10/2669.full http://www.fsis.usda.gov/factsheets/Bovine_Spongiform_Encephalopathy_Mad_Cow_Disease/index.asp Other Prion Diseases. Creutzfeldt-Jakob disease (CJD) is a rare, degenerative, invariably fatal brain disorder. In the early stages of disease, people may have failing memory, behavioral changes, lack of coordination and visual disturbances. As the illness progresses, mental deterioration becomes pronounced and involuntary movements, blindness, weakness of extremities, and coma may occur. http://www.ninds.nih.gov/disorders/cjd/detail_cjd.htm Kuru is a rare and fatal brain disorder, which was the result of the practice of ritualistic cannibalism (of where relatives prepared and consumed the tissues, including brain of deceased family members.) Brain tissue from individuals with kuru was highly infectious, and the disease was transmitted either through eating or by contact with open sores or wounds. Government discouragement of the practice of cannibalism led to a continuing decline in the disease, which has now mostly disappeared. http://www.ninds.nih.gov/disorders/kuru/kuru.htm Scrapie is a disease of sheep and goats that destroys their brains. The disease came from Great Britain in 1947. It kills the brain cells of sheep so that they can not get up to eat and drink. Animals that have scrapie will rub against things and scrape off their wool or hair. This scraping by the animals is what led to the naming of the disease. Currently there is no cure for scrapie There is no true use of prions in medicine because it is found normally in the body or can be very harmful in the wrong form. But studies do show that prions expressions on stem cells is necessary for the body's renewal of bone marrow Use of prions in medicine http://peds.oxfordjournals.org/content/13/2/99.full Normal prions are pivotal in memory processing and have a major role in the nervous system. Studies shows a defficiency in PrP in the brain may lead to decreased motor coordination as one ages. prions are transmitted by ingestion of infected meats or from environmental deposits from dead animals and urine.
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