http://www.sciencedaily.com/news/plants_animals/prions/ Learn all about prions and prion disease. How are prions linked to mad cow disease, TSE, Creutzfeldt-Jacob disease and chronic wasting disease? en-us Thu, 12 Nov 2009 00:05:01 EST Thu, 12 Nov 2009 00:05:01 EST 60 http://www.sciencedaily.com/images/logosmall.gif http://www.sciencedaily.com/news/plants_animals/prions/ For more science articles, visit ScienceDaily. http://www.sciencedaily.com/releases/2009/10/091005161324.htm Scientists have discovered the first direct information about the molecular structure of prions. In addition, the study has revealed surprisingly large structural differences between natural prions and the closest synthetic analogs that scientists have created in the lab. Tue, 06 Oct 2009 02:00:00 EDT http://www.sciencedaily.com/releases/2009/10/091005161324.htm http://www.sciencedaily.com/releases/2009/09/090928131210.htm Scientists have uncovered the evolutionary ancestry of the prion gene, which may reveal new understandings of how the prion protein causes diseases such as bovine spongiform encephalopathy, also known as "mad cow disease." Tue, 29 Sep 2009 11:00:00 EDT http://www.sciencedaily.com/releases/2009/09/090928131210.htm http://www.sciencedaily.com/releases/2009/09/090910191443.htm A new study suggests that gene expression profiling may allow researchers to track the progression of bovine spongiform encephalopathy (BSE) in cattle and ultimately predict their infectious status. Thu, 17 Sep 2009 02:00:00 EDT http://www.sciencedaily.com/releases/2009/09/090910191443.htm http://www.sciencedaily.com/releases/2009/08/090826152550.htm Researchers have shown definitively that mutations associated with prion diseases are sufficient to cause a transmissible neurodegenerative disease. Until now, two theories about the role mutations play in prion diseases have been at odds. Deciphering the origins of prion diseases could help farmers and policy-makers determine how best to control a prion disease outbreak in livestock and to prevent prion transmission to humans. Thu, 27 Aug 2009 23:00:00 EDT http://www.sciencedaily.com/releases/2009/08/090826152550.htm http://www.sciencedaily.com/releases/2009/07/090730111152.htm Data from an ongoing study in monkeys suggest that people who consume deer and elk with chronic wasting disease may be protected from infection by an inability of the CWD infectious agent to spread to people. Mon, 03 Aug 2009 23:00:00 EDT http://www.sciencedaily.com/releases/2009/07/090730111152.htm http://www.sciencedaily.com/releases/2009/06/090625141506.htm Researchers have discovered a link between copper and the normal functioning of prion proteins, which are associated with transmissible spongiform encephalopathy diseases such as Cruetzfeldt-Jakob in humans or "mad cow" disease in cattle. Their work could have implications for patients suffering from these diseases, as well as from other prion-related diseases such as Alzheimers or Parkinson's. Sun, 28 Jun 2009 11:00:00 EDT http://www.sciencedaily.com/releases/2009/06/090625141506.htm http://www.sciencedaily.com/releases/2009/06/090616080143.htm Neurologists questions the safety of eating farmed fish, adding a new worry to concerns about the nation’s food supply. They suggest farmed fish could transmit Creutzfeldt Jakob disease -- commonly known as mad cow disease -- if they are fed byproducts rendered from cows. Wed, 17 Jun 2009 20:00:00 EDT http://www.sciencedaily.com/releases/2009/06/090616080143.htm http://www.sciencedaily.com/releases/2009/05/090528203819.htm Current tests to identify specific strains of infectious prions, which cause a range of transmissible diseases (such as mad cow) in animals and humans, can take anywhere from six months to a year to yield results -- a time-lag that may put human populations at risk. Mon, 01 Jun 2009 14:00:00 EDT http://www.sciencedaily.com/releases/2009/05/090528203819.htm http://www.sciencedaily.com/releases/2009/04/090419201836.htm Goats are tough, spirited animals, but they're no match for scrapie, a form of transmissible spongiform encephalopathy. Now, with a "helping hand" from science, the animals' plight could take a turn for the better. Sun, 26 Apr 2009 05:00:00 EDT http://www.sciencedaily.com/releases/2009/04/090419201836.htm http://www.sciencedaily.com/releases/2009/04/090417084124.htm An investigation of a rare, inherited form of Creutzfeldt-Jakob disease suggests that disrupted regulation of copper ions in the brain may be a key factor in this and other prion diseases. Mon, 20 Apr 2009 02:00:00 EDT http://www.sciencedaily.com/releases/2009/04/090417084124.htm http://www.sciencedaily.com/releases/2009/04/090402124320.htm Special proteins known as prions, which are perhaps best known as the agents of mad cow and other neurodegenerative diseases, can also serve as an important source of beneficial variation in nature. Researchers have found a large number of new prions, greatly expanding scientists' notion of how important prions might be in normal biology and demonstrating that they play many and varied roles in the inheritance of biological traits. Prions are misfolded proteins that clump together in cells. The most infamous known prion -- PrP -- causes bovine spongiform encephalitis, also known as "mad cow" disease. Tue, 07 Apr 2009 14:00:00 EDT http://www.sciencedaily.com/releases/2009/04/090402124320.htm http://www.sciencedaily.com/releases/2009/03/090313171316.htm The discovery of a new yeast prion may provide clues on whether prions, like proteins, can affect mass activation of gene expression. Fri, 20 Mar 2009 20:00:00 EDT http://www.sciencedaily.com/releases/2009/03/090313171316.htm http://www.sciencedaily.com/releases/2009/03/090313145954.htm Imbalance of iron homeostasis is a common feature of prion disease-affected human, mouse, and hamster brains, according to a new study. These findings provide new insight into the mechanism of neurotoxicity in prion disorders, and novel avenues for the development of therapeutic strategies. Fri, 20 Mar 2009 05:00:00 EDT http://www.sciencedaily.com/releases/2009/03/090313145954.htm http://www.sciencedaily.com/releases/2009/03/090309205323.htm Scientists have known for some time that a normal protein in the brain, prion protein (PrP), can turn harmful and cause deadly illnesses like CJD in humans, and BSE in cattle. What they could not explain is why this normal protein is produced by our bodies. Sun, 15 Mar 2009 05:00:00 EDT http://www.sciencedaily.com/releases/2009/03/090309205323.htm http://www.sciencedaily.com/releases/2009/02/090210092736.htm Harmful proteins fragments known as amyloid fibrils associated with damage to brain cells in Alzheimer's disease and to pancreatic cells in Type II diabetes can be present in the meat of poultry and mammals. These amyloids are not destroyed even with high-temperature cooking process. Thu, 19 Feb 2009 08:00:00 EST http://www.sciencedaily.com/releases/2009/02/090210092736.htm http://www.sciencedaily.com/releases/2009/01/090127214430.htm Scientists have created a new disease, comparable to BSE, in laboratory mice. They have shown that exchanging just two amino acids in the structure of the prion protein is enough to trigger a disease. Fri, 30 Jan 2009 14:00:00 EST http://www.sciencedaily.com/releases/2009/01/090127214430.htm http://www.sciencedaily.com/releases/2009/01/090109095115.htm Scrapie is a transmissible, degenerative and ultimately fatal disease of the nervous system of sheep. The cause of the disease is a prion protein, and absorption from the intestine is assumed to be the natural route of infection. Lymphatic tissue associated with the intestine is important for the early accumulation of prion protein and its subsequent spread to the central nervous system. Sat, 17 Jan 2009 14:00:00 EST http://www.sciencedaily.com/releases/2009/01/090109095115.htm http://www.sciencedaily.com/releases/2008/12/081205094509.htm Researchers have found novel prion infectivity in white and brown fat tissues of mice. Prion diseases, also known as transmissible spongiform encephalopathies, are infectious progressive fatal neurodegenerative diseases which affect humans as well as wild and domestic animals. Mon, 08 Dec 2008 23:00:00 EST http://www.sciencedaily.com/releases/2008/12/081205094509.htm http://www.sciencedaily.com/releases/2008/12/081205122936.htm Scientists have created an infectious prion disease in a mouse model, in a step that may help unravel the mystery of this progressive disease that affects the nervous system in humans and animals. Sat, 06 Dec 2008 20:00:00 EST http://www.sciencedaily.com/releases/2008/12/081205122936.htm http://www.sciencedaily.com/releases/2008/11/081126133300.htm A comprehensive mouse model of inherited prion disease exhibits cognitive, motor and neurophysiological deficits that bear a striking resemblance to the symptoms experienced by patients with the human version of "mad cow disease," Creutzfeldt-Jakob disease. The research, published in the journal Neuron, provides exciting insight into the mechanism of disease and may lead to the development of new therapeutic strategies for this devastating neurodegenerative disorder. Fri, 28 Nov 2008 20:00:00 EST http://www.sciencedaily.com/releases/2008/11/081126133300.htm http://www.sciencedaily.com/releases/2008/10/081008113430.htm Researchers in Germany and Switzerland have developed a new general method for the synthesis of anchored proteins, such as GPI-anchored prions, which cause scrapie and mad cow disease. Fri, 10 Oct 2008 08:00:00 EDT http://www.sciencedaily.com/releases/2008/10/081008113430.htm http://www.sciencedaily.com/releases/2008/09/080929212926.htm Prion protein, a form of protein that triggers BSE, is associated with other brain diseases in cattle, raising the possibility of a significant increase in the range of prion disease. Scientists have detected changes in the production and accumulation of the prion protein in the brains of cattle with a rare neurodegenerative disorder. Tue, 30 Sep 2008 17:00:00 EDT http://www.sciencedaily.com/releases/2008/09/080929212926.htm http://www.sciencedaily.com/releases/2008/09/080912075208.htm New findings about the causes of mad cow disease show that sometimes it may be genetic. Until several years ago, it was thought that the cattle prion disease bovine spongiform encephalopathy -- also called BSE or mad cow disease -- was a foodborne disease. Sat, 13 Sep 2008 20:00:00 EDT http://www.sciencedaily.com/releases/2008/09/080912075208.htm http://www.sciencedaily.com/releases/2008/09/080905153643.htm Researchers have demonstrated that protein levels in urine samples can indicate both the presence and progress of Bovine Spongiform Encephalopathy disease in cattle. The scientists hope that their discovery might lead to the development of a urine-based test that could prevent the precautionary slaughter of many animals as now occurs when the disease is detected. Mon, 08 Sep 2008 11:00:00 EDT http://www.sciencedaily.com/releases/2008/09/080905153643.htm http://www.sciencedaily.com/releases/2008/09/080904144830.htm Researchers have shown that they can create entirely new strains of infectious proteins known as prions in the laboratory by simply mixing infectious prions from one species with the normal prion proteins of another species. Fri, 05 Sep 2008 14:00:00 EDT http://www.sciencedaily.com/releases/2008/09/080904144830.htm http://www.sciencedaily.com/releases/2008/08/080829104935.htm A nine-year study in sheep has added to the evidence that Creutzfeldt-Jakob disease (vCJD) can be transmitted through blood transfusion in humans. The likelihood of Bovine spongiform encephalopathy (BSE) being transmitted between sheep through transfusion of infected sheep blood was 36 per cent, according to new research. Mon, 01 Sep 2008 14:00:00 EDT http://www.sciencedaily.com/releases/2008/08/080829104935.htm http://www.sciencedaily.com/releases/2008/08/080828220507.htm Scientists have identified a prion protein characteristic that is unique to some natural but unusual sheep scrapie cases. This finding may provide a novel method by which to study prion diversity and their possible changes during cross-species transmission. Sat, 30 Aug 2008 14:00:00 EDT http://www.sciencedaily.com/releases/2008/08/080828220507.htm http://www.sciencedaily.com/releases/2008/08/080811095458.htm Prions, the pathogens that cause scrapie in sheep, can survive in the ground for several years, as researchers have discovered. Animals can become infected via contaminated pastures. It is not yet known whether the pathogens that cause BSE and CWD are equally resistant. Sat, 16 Aug 2008 17:00:00 EDT http://www.sciencedaily.com/releases/2008/08/080811095458.htm http://www.sciencedaily.com/releases/2008/08/080811072501.htm Abnormally folded proteins cause a number of illnesses such as the Creutzfeldt-Jacob Disease, BSE (bovine spongiform encephalopathy) and Alzheimer’s. It is still unknown why this misfolding occurs. The first stages of folding and the onset of the aggregation of the proteins, the so-called oligomerisation, appear to be decisive for pathogenesis. Mon, 11 Aug 2008 23:00:00 EDT http://www.sciencedaily.com/releases/2008/08/080811072501.htm http://www.sciencedaily.com/releases/2008/06/080617111831.htm Infectious proteins known as prions have been identified as the cause of “mad cow” disease (BSE). The culprits are “incorrectly folded” proteins that can “infect” healthy proteins. The molecular bases for such prion diseases are not yet fully understood. Why are some proteins infectious while others are not? Scientists examined two different forms of a prion-forming protein domain by means of NMR spectroscopy and found that the infectious and noninfectious forms differ markedly in their molecular structure. Thu, 19 Jun 2008 20:00:00 EDT http://www.sciencedaily.com/releases/2008/06/080617111831.htm http://www.sciencedaily.com/releases/2008/05/080507105649.htm Prions, the infamous agents behind mad cow disease and its human variation, Creutzfeldt-Jakob disease, also have a helpful side. New research shows that normally functioning prions prevent neurons from working themselves to death. The findings appear in the May 5 issue of the Journal of Cell Biology. Fri, 09 May 2008 05:00:00 EDT http://www.sciencedaily.com/releases/2008/05/080507105649.htm http://www.sciencedaily.com/releases/2008/04/080408144827.htm Amid concern that recipients of certain blood transfusions may risk infection with a deadly protein responsible for the human form of mad cow disease, researchers now report development of a special filter that quickly and effectively removes the protein from blood. Sat, 12 Apr 2008 08:00:00 EDT http://www.sciencedaily.com/releases/2008/04/080408144827.htm http://www.sciencedaily.com/releases/2008/04/080407190603.htm Scrapie can be transmitted to lambs through milk, according to new research. The study provides important information on the transmission of this prion-associated disease and the control of scrapie in affected flocks. Tue, 08 Apr 2008 14:00:00 EDT http://www.sciencedaily.com/releases/2008/04/080407190603.htm http://www.sciencedaily.com/releases/2008/03/080317154402.htm Researchers have identified four separate biochemical subgroups in a selection of cases of Creutzfeldt-Jakob disease. The study suggests that these subgroups could represent distinct prion strains in what is the most common human prion disease. Wed, 19 Mar 2008 08:00:00 EDT http://www.sciencedaily.com/releases/2008/03/080317154402.htm http://www.sciencedaily.com/releases/2008/03/080303093549.htm In an advance in food safety, researchers are reporting development of a nano-sized sensor that detects record low levels of the deadly prion proteins that cause Mad Cow Disease and other so-called prion diseases. The sensor, which detects binding of prion proteins by detecting frequency changes of a micromechanical oscillator, could lead to a reliable blood test for prion diseases in both animals and humans, the researchers say. Wed, 05 Mar 2008 14:00:00 EST http://www.sciencedaily.com/releases/2008/03/080303093549.htm http://www.sciencedaily.com/releases/2007/12/071204154723.htm Scientists have developed two new tests for prions, infectious proteins that cause a number of diseases including "mad cow disease," and a human counterpart, variant Creutzfeldt-Jakob disease. These advances open the door to better understanding and diagnosis of these troubling conditions. Wed, 12 Dec 2007 11:00:00 EST http://www.sciencedaily.com/releases/2007/12/071204154723.htm http://www.sciencedaily.com/releases/2007/11/071130144506.htm Rapid diagnostic testing used to check for the presence of prion diseases such as "mad cow disease" might fail to identify some highly infectious samples, researchers have found. Sat, 01 Dec 2007 20:00:00 EST http://www.sciencedaily.com/releases/2007/11/071130144506.htm http://www.sciencedaily.com/releases/2007/11/071121221704.htm An effective and sensitive new method for detecting and characterizing prions, the infectious compounds behind diseases like mad cow disease, is now being launched. Mad cow disease (BSE), which has caused the death of more than 200,000 cattle and 165 people in the U.K., has now abated. But other prion disorders are on the rise, and there is concern that new strains will infect humans. Thu, 22 Nov 2007 14:00:00 EST http://www.sciencedaily.com/releases/2007/11/071121221704.htm http://www.sciencedaily.com/releases/2007/09/070926192035.htm Lymph nodes can be crucial for spreading low doses of infective prion agents -- the pathogens responsible for conditions such as scrapie and Creutzfeldt-Jakob disease -- into the nervous system, according to new research. Mon, 01 Oct 2007 11:00:00 EDT http://www.sciencedaily.com/releases/2007/09/070926192035.htm http://www.sciencedaily.com/releases/2007/09/070907095625.htm Prion proteins may activate endogenous retroviruses in infected brain cells. Prions – an abbreviation for proteinaceous infectious particles – work as a trigger to a set of diseases of the brain and nervous system, the so-called spongiform encephalopathies. These include BSE in cattle, scrapie in sheep and Creutzfeldt Jakob’s Disease in humans. Prions are structural variants of a normal protein found in healthy tissues – especially in the brain. Sat, 15 Sep 2007 17:00:00 EDT http://www.sciencedaily.com/releases/2007/09/070907095625.htm http://www.sciencedaily.com/releases/2007/09/070905152439.htm Scientists have shown for the first time that small clumps of abnormal prion proteins called oligomers cause the widespread death of neurons. In contrast, much larger prion aggregates known as fibrils proved to be far less toxic. The findings suggest that small protein aggregates play a central role in prion diseases; similar mechanisms have been proposed for the so-called "amyloid" neurodegenerative diseases, including Alzheimer's. The work may provide novel therapeutic approaches for treating people with these conditions. Mon, 10 Sep 2007 23:00:00 EDT http://www.sciencedaily.com/releases/2007/09/070905152439.htm http://www.sciencedaily.com/releases/2007/08/070816121102.htm Scientists have discovered a new protein that may offer fresh insights into brain function in mad cow disease. Fri, 17 Aug 2007 11:00:00 EDT http://www.sciencedaily.com/releases/2007/08/070816121102.htm http://www.sciencedaily.com/releases/2007/07/070705094120.htm Scientists have made significant advances towards the development of a technique that could be used to confirm whether someone is infected with variant Creutzfeldt-Jakob Disease. Tue, 10 Jul 2007 05:00:00 EDT http://www.sciencedaily.com/releases/2007/07/070705094120.htm http://www.sciencedaily.com/releases/2007/07/070706081324.htm The rogue proteins that cause chronic wasting disease (CWD) exhibit a dramatic increase in their infectious nature when bound to common soil particles, according to a new study. Mon, 09 Jul 2007 02:00:00 EDT http://www.sciencedaily.com/releases/2007/07/070706081324.htm http://www.sciencedaily.com/releases/2007/07/070705121847.htm Using baker's yeast and another fungus, researchers report the first successful propagation of a prion from one organism to another. Prions -- infectious, oddly-folded proteins that are the main suspects in fatal neurodegenerative diseases such as Cruetzfeldt-Jakob and bovine spongiform encephalopathy, or "mad cow" -- remain mostly a mystery to scientists. Fri, 06 Jul 2007 17:00:00 EDT http://www.sciencedaily.com/releases/2007/07/070705121847.htm http://www.sciencedaily.com/releases/2007/07/070702145402.htm Sheep infected with scrapie and cows infected with BSE have elevated levels of manganese in their blood before clinical symptoms appear, according to new research. Wed, 04 Jul 2007 20:00:00 EDT http://www.sciencedaily.com/releases/2007/07/070702145402.htm http://www.sciencedaily.com/releases/2007/05/070509161210.htm Prions are highly robust and infectious proteins, most notable for their central role in bovine spongiform encephalopathy, commonly called mad cow disease. But very little is known about how prions form aggregates of malformed proteins that ultimately result in disease. This study provides initial insights into how prions recruit and distort healthy neighboring proteins. Wed, 09 May 2007 23:00:00 EDT http://www.sciencedaily.com/releases/2007/05/070509161210.htm http://www.sciencedaily.com/releases/2007/05/070503135420.htm An oral vaccine can prevent mice from developing a brain disease similar to mad cow disease, according to new research. Prion diseases, which include scrapie, mad cow disease and chronic wasting disease, are fatal, and there is no treatment or cure. Thu, 03 May 2007 20:00:00 EDT http://www.sciencedaily.com/releases/2007/05/070503135420.htm