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 prions

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updated Sat. April 28, 2018

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Prions are an incredibly bizarre type of pathogen that are even further removed from the concept of life than viruses. They're the misshapen form of proteins already abundant in the brain. But when a prion comes into contact with its healthy counterpart, it somehow forces the normal protein to become ...
CWD is a type of brain-damaging and fatal prion disease found in deer, elk and moose; in humans, prion diseases can take more than a decade to develop. In the study, appearing in the Journal of Virology, 14 macaques were cerebrally and orally exposed to brain matter from CWD-infected deer and ...
Chronic wasting disease (CWD) did not cross the species barrier to infect cynomolgus macaque monkeys during a lengthy investigation by National Institutes of Health scientists exploring risks to humans. CWD is a type of brain-damaging and fatal prion disease found in deer, elk and moose; in ...
Generally, prions are infectious, deformed versions of the normal prion protein (PRNP), which functions on the outside of healthy cells in the brain. The gnarled versions prompt normal prion proteins to deform and malfunction, setting off a cascade that leads to telltale clumps of contorted proteins. This ... Africa
Dr. Roos said procedures such as lumbar punctures in patients believed to have prion disease should only be attended by personnel who are familiar with prion diseases and respect their dangers. He said that prions can survive on surfaces for long periods of time and therefore their surfaces used ... Reporter

"Our identification of this prion disease in a geographically widespread livestock species requires urgent enforcement of surveillance and assessment of ... had first been alerted to disease in camels when Algerian researchers observed symptoms in the animals reminiscent of other prion diseases such ...
"These camels are quite intriguing," prion expert Valerie Sim, MD, associate professor at the University of Alberta, told CIDRAP News. "If we know anything about prions it's that they can they can cross species; it's not easy to do, but they can. So it's very concerning if you have any infected animals in ...
This, at least, is the prion hypothesis as promulgated by biologist Stanley Prusiner, who won the Nobel Prize in Medicine in 1997 for the idea. The ensuing chain reaction drives a relentless conversion of normal prion proteins into prions. In many prion diseases, the shape of the prion also drives them ...
Montanans took note this past year of prion-based diseases when Chronic Wasting Disease was discovered in deer herds in eastern Montana. ... If infectious prions could survive that long in a sterile environment, it's easier to understand how they might remain viable in the soil long enough to impact ...
Prion diseases are progressive nervous system diseases which always have a fatal outcome. They are transmissible and affect many mammals. They are also called transmissible spongiform encephalopathies (TSEs). Their root disorder is the abnormal tertiary folding of a protein encoded by the host cells ...
Prions are the misfolded versions of the prion protein that can attack the brain from the inside and cause a variety of neurodegenerative diseases, including Creutzfeldt-Jakob disease (CJD). Now a new study makes them even scarier than before: they can lurk undetected for 30 years before attacking.
Familial human prion diseases are passed within families and are associated with 34 known prion protein mutations. To determine whether three of the unstudied mutations are transmissible, scientists from the National Institute of Allergy and Infectious Diseases (NIAID), part of the National Institutes of ...
When prions are next to their normal counterparts, they very slowly, but relentlessly, convert them into more prions, like a microscopic Agent Smith from the Matrix. Little by little, the growing army eats away at the brain, causing telltale spongy holes. It can take years, even decades for a prion disease to ...
Familial human prion diseases are passed within families and are associated with 34 known prion protein mutations. To determine whether three of the unstudied mutations are transmissible, scientists from the National Institute of Allergy and Infectious Diseases, part of the National Institutes of Health, ...
Chronic wasting disease is caused by a prion, a sort of mutant protein with an unusual folding pattern that tricks the body's own proteins into mutating too. ... "Clay can tend to immobilize molecules, and we think at these higher concentrations, clay is holding onto the prions, so they're not bioavailable," said ...
CWD is part of a family of diseases known as transmissible spongiform encephalopathies or prion diseases. Other TSEs include bovine ... CWD is invariably fatal in cervids, and is believed to be transmitted through prions, which are misfolded proteins that attack the nervous system of the host. These prions ...

MacGillivray's Prions (Pachyptila macgillivrayi) breed in Prion Cave on Gough Island from November though to April and during this time the Gough field team make regular visits to Prion Cave to monitor their success. The data collected this season, like many before, shows the catastrophic effect mice have ...
“When infected deer urinate, defecate, or salivate, they can shed prions. When a susceptible deer comes along and licks, ingests, or inhales infected soil, it could pick up a prion. But soil is complex. It's not clear what soil characteristics are associated with the persistence of chronic wasting disease in deer,” ...
... of dead, infected deer can also contaminate the soil. Plants growing in that soil can take up the prions. Deer can become infected by feeding in areas with prion-contaminated soil and plants. The prions remain in the environment for years. There is no practical method of decontaminating an infected area.
National Institutes of Health scientists developing a rapid, practical test for the early diagnosis of prion diseases have modified the assay to offer the possibility of improving early diagnosis of Parkinson's disease and dementia with Lewy bodies. The group, led by NIH's National Institute of Allergy and ...
"When infected deer urinate, defecate, or salivate, they can shed prions. When a susceptible deer comes along and licks, ingests, or inhales infected soil it could pick up a prion. But soil is complex. It's not clear what soil characteristics are associated with the persistence of chronic wasting disease in deer," ...
According to Chernoff, yeast also form prions, and the initial nucleation of a yeast prion is also rare. “However,” he says, “it is easier to detect prion nucleation in yeast that in humans, because it is possible to analyze large numbers of yeast cells, and because yeast prions cause easily detectable traits.”.
Nova say that the new Prion 4 makes learning to fly simple, and is a joy to fly long after your training days. They note that previously EN-A wings were nice safe wings to learn on in school, but they would tend to be traded in for “proper”, more fun wings as soon as training was over. Nova say the Prion 4 ...
Scientists who are developing a rapid, practical test for the early diagnosis of prion diseases have modified the test to offer the possibility of improving early ... Like prion diseases, Parkinson's disease and dementia with Lewy bodies result in progressive deterioration of brain functions and, ultimately, death.
For those unfamiliar with it, I'll explain: Creutzfeldt-Jakob disease is the most common prion disease in humans. (The others are predominantly found outside the United States.) Prions are infectious, but they're not viruses or bacteria; they're proteins. They appear to be created from normal proteins found on ...
They found that all four people had undergone neurosurgery two or three decades earlier as children or teenagers, raising the possibility that amyloid beta deposition may be transmissible through neurosurgical instruments in a similar way to prion proteins which are implicated in prion dementias such as ...
CWD, which is spread by misfolded proteins known as prions,hasn't been known to infect people. But researchers have warned that it could. ... A primary reason that officials are worried comes from the preliminary results of an ongoing study by the Alberta Prion Research Institute. The findings showed that ...
"When infected deer urinate, defecate, or salivate, they can shed prions. When a susceptible deer comes along and licks, ingests, or inhales infected soil it could pick up a prion. But soil is complex. It's not clear what soil characteristics are associated with the persistence of chronic wasting disease in deer," ...
The fairy prion population is at about 1.8 million pairs, he said. "Even if the young ones are not in good condition, and getting washed up, it's not having an overall bad effect on the population. "It's one of these things that if every prion that fledged survived, the world would be covered in prions. While its ...
According to Chernoff, yeast also form prions, and the initial nucleation of a yeast prion is also rare. “However,” he says, “it is easier to detect prion nucleation in yeast that in humans, because it is possible to analyze large numbers of yeast cells, and because yeast prions cause easily detectable traits.”.
While most animals usually do not contract prions from other species, the bank vole easily contracts defective prions from other animals, Glynn said. Marcus Gallagher-Jones, a postdoctoral researcher in Rodriguez's lab, said studying the bank vole prion may explain why bank voles are so susceptible to ...
... the tribes of New Guinea that is spread by cannibalism. Prions are infectious proteins and, at the time Prusiner first announced his findings, much of the scientific community expressed doubts that simple proteins could be infectious agents. A lot of very smart people scoffed at the notion of a prion disease.
... the tribes of New Guinea that is spread by cannibalism. Prions are infectious proteins and, at the time Prusiner first announced his findings, much of the scientific community expressed doubts that simple proteins could be infectious agents. A lot of very smart people scoffed at the notion of a prion disease.
“Different prion diseases tend to only harm certain species, but can evolve to overcome those limitations.” In some herds, as many as half of the animals carry prions. But direct contact isn't the only way prions are transmitted. According to The New York Times, sick animals and cadavers can spread prions ...
Mad cow disease, for example, is a prion disease that rooted from scrapie, a deadly disease that afflicts sheep. Once the prions were passed to cows, the cows developed a prion disease of their own (mad cow disease). And when humans ate the beef from those sick cows, they developed prions in their ...
For those unfamiliar with it, I'll explain: Creutzfeldt-Jakob disease (CJD) is the most common prion disease in humans. (The others are predominantly found outside the United States.) Prions are infectious, but they're not viruses or bacteria; they're proteins. They appear to be created from normal proteins ...
That evidence, or lack of it, suggests a strong "species barrier" between deer and humans. CWD passes from animal to animal through prions, misfolded proteins that cause other proteins to misfold around them. Different prion diseases tend to only harm certain species, but can evolve to overcome those ...
Dr. Caughey added: “Our data show that there are prions in the skin but they are at a very low level, and there is no evidence that these levels represent a transmission risk under practical clinical circumstances. We also don't know what part of the skin contains this prion seeding activity.” The researchers ...
Dr. Caughey added: “Our data show that there are prions in the skin but they are at a very low level, and there is no evidence that these levels represent a transmission risk under practical clinical circumstances. We also don't know what part of the skin contains this prion seeding activity.” The researchers ...
Worse, instead of being destroyed and recycled by the cell -- the typical fate of misfolded proteins -- prions resist degradation and cause other normal prion proteins to misfold. A slow chain reaction occurs in the brain over the course of many years, leading to neurodegenerative disease and death.
Indeed, pathological prions cause mad cow disease and in humans Creutzfeldt-Jakob disease. The aggregation of prion-like proteins is also associated with neurodegeneration as in ALS. The regions within prion-like proteins that are responsible for their aggregation were termed prion-like domains.
Much recent work has focused on liquid-liquid phase separation as a cellular response to changing physicochemical conditions. Because phase separation responds critically to small changes in conditions such as pH, temperature, or salt, it is in principle an ideal way for a cell to measure and respond to ...
... of dead, infected deer can also contaminate the soil. Plants growing in that soil can take up the prions. Deer can become infected by feeding in areas with prion-contaminated soil and plants. The prions remain in the environment for years. There is no practical method of decontaminating an infected area.
With a cast and crew in the triple digits, and a run-time to match, the 26th Annual Addenbrooke's Charity Pantomime, The Prion King—and its Cambridge School of Clinical Medicine student writers, producers, and performers—delivers plenty of colourful laughs, even for those whose only experience with ...
“Macaques are susceptible to human prions, and that's why they're a good model for a species barrier jump,” Mark Zabel of Colorado State University's Prion Research Center told Newsweek. “We know that they will develop a prion disease from human prions.” Zabel was not involved in the macaque study.
In prion diseases, an abnormal isoform of prion protein (PrPSc) accumulates in neurons, astrocytes, and microglia in the brains of animals affected by prions. Detailed analyses of PrPSc-positive neurons and glial cells are required to clarify their pathophysiological roles in the disease. Here, we report a ...
... pathogenic event in prion diseases, including Creutzfeldt-Jakob disease in humans and scrapie and bovine spongiform encephalopathy (BSE) in animals. We previously reported that the octapeptide repeat (OR) region could be dispensable for converting PrPC into PrPSc after infection with RML prions.
Sporadic Creutzfeldt–Jakob disease (sCJD) can be transmitted via surgical tools that are contaminated with misfolded infectious prion protein (PrPSc) during surgeries that involve the CNS. For reasons that are unclear, some reports have found an epidemiological association between sCJD and non-CNS ...
Infectious prions are detectable in the skin samples of patients with sporadic Creutzfeldt-Jakob disease (sCJD), according to a study published online on November 22 in Science Translational Medicine. Previous studies have shown that sCJD, the most common prion disease in humans, can be transmitted ...


 

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