Prion

Fibril Shape Is The Basis Of Prion Strains And Cross-species Prion Infection

New research on prions, the infectious proteins behind "mad cow" disease and Creutzfeld-Jakob disease in humans, suggests that the ability of prions in one species to infect other species depends on the shape of the toxic threadlike fibers produced by the prion. Two studies on the topic appear in the 8 April issue of the journal Cell. Although research suggests that prions from one specie...

Strongest proof yet found for prion hypothesis

UTMB scientists offer strongest evidence yet that infectious misformed proteins cause mad cow disease and other mysterious brain disorders Researchers at the University of Texas Medical Branch at Galveston (UTMB) have produced the strongest proof yet that the mysterious and devastating brain diseases known as "transmissible spongiform encephalopathies" (TSEs) are transmitted by an infecti...

First technology to remove prions that cause vCJD from blood launched

The risk of receiving blood contaminated with variant Creutzfeldt-Jakob (vCJD) prions may no longer be a concern for the thousands of people who require a transfusion. Pall Corporation (NYSE: PLL) announced today the Council of Europe (CE) marking of its Leukotrap® Affinity Prion Reduction Filter System. It is the first and only technology that removes infectious prions that may be the causative...

Research into how prions act in the brain could hold the key to defeating diseases

Scientists at the University of Southampton are continuing their pioneering research into diseases such as Creutzfeld-Jakob Disease (CJD) and related conditions caused by tiny particles known as prions, thanks to a £300,000 grant from the Medical Research Council. So far, there have been 148 deaths from confirmed or probable variant CJD (vCJD) in the UK. This is the human disease thought...

Active Vaccine Prevents Mice From Developing Prion Disease

NYU School of Medicine scientists have created the first active vaccine that can significantly delay and possibly prevent the onset of a brain disease in mice that is similar to mad cow disease. The new findings, published online this week in the journal Neuroscience, could provide a platform for the development of a vaccine to prevent a group of fatal brain diseases caused by unusual infectious...

Unweaving amyloid fibers to solve prion puzzles

Amyloid fibers are best known as the plaque that gunks up neurons in people with neurodegenerative illnesses such as Alzheimer's and Creutzfeldt-Jacob disease--the human analog of mad cow disease. But even though amyloids are common and implicated in a host of conditions, researchers haven't been able to identify their precise molecular structures. Conventional techniques used to image proteins,...

Variant prion protein causes infection but no symptoms

Abnormal prion proteins are little understood disease agents involved in causing horrific brain-wasting diseases such as Creutzfeldt-Jacob disease in people, mad cow disease in cattle and chronic wasting disease in deer and elk. Now, new research suggests that a variant form of abnormal prion protein--one lacking an "anchor" into the cell membrane--may be unable to signal cells to start the letha...

New results validating Pall prion reduction filter disclosed at ISBT Congress

Safety in transfusion medicine is an ongoing challenge to blood services worldwide as new threats, such as variant Creutzfeldt-Jakob Disease (vCJD), emerge. An update on the latest measures to protect the public from the human form of Bovine Spongiform Encephalopathy (BSE), commonly called Mad Cow Disease, and minimize risk of transmission through blood transfusion was presented here at the Inter...

Prions rapidly 'remodel' good protein into bad, Brown study shows

Two Brown Medical School biologists have figured out the fate of healthy protein when it comes in contact with the infectious prion form in yeast: The protein converts to the prion form, rendering it infectious. In an instant, good protein goes bad. This quick-change "mating" maneuver sheds important light on the mysterious molecular machinery behind prions, infectious proteins that cause...

New evidence questions the simple link between prion proteins and madcow disease

While newly published research confirms that under laboratory circumstances prion-protein can be absorbed across the gut, it also shows that this is unlikely to occur in real life. In addition, the results show that the places in the gut that do take up these disease-associated proteins are different from the locations where infectivity is known to be amplified. The findings will be published in...

Soil-bound prions that cause CWD remain infectious

Researchers discovered a new bacterium in an immune-compromised patient, according to a study recently published in PLoS Pathogens. The bacterium belongs to the family Acetobacteraceae and includes bacteria common in the environment, some of which are used in industry, such as vinegar-making. "This is the first reported case of invasive human disease caused by any of the Acetobacteraceae," acco...

Brittle prions are more infectious

Brittleness is often seen as a sign of fragility. But in the case of infectious proteins called prions, brittleness makes for a tougher, more menacing pathogen. Howard Hughes Medical Institute researcher have discovered that brittle prion particles break more readily into new "seeds," which spread infection much more quickly. The discovery boosts basic understanding of prion infections, an...

Prion find points way to test for human 'mad cow' disease

In the July 7, 2006, issue of the journal Science, researchers at the University of Texas Medical Branch at Galveston (UTMB) describe experiments that may soon lead to a test that will enable medical science to estimate how many people are infected with the human form of mad cow disease, which can take as long as 40 years before manifesting itself. Such a blood test could also help preven...

Prion disease treatable if caught early

Studies in mice have indicated that the effects of prion disease could be reversed if caught early enough. The researchers said that their findings support developing early treatments that aim to reduce levels of prion protein in the brains of people with prion disease. Also, they said that their findings suggest testing the efficacy of treatments in a new way: by analyzing their cognitive effect...

Brown team finds crucial protein role in deadly prion spread

A single protein plays a major role in deadly prion diseases by smashing up clusters of these infectious proteins, creating the "seeds" that allow fatal brain illnesses to quickly spread, new Brown University research shows. The findings are exciting, researchers say, because they might reveal a way to control the spread of prions through drug intervention. If a drug could be made that inh...

Scripps research study reveals structural dynamics of single prion molecules

The new findings, which are being published the week of February 12 in an online edition of the Proceedings of the National Academy of Sciences, offer significant insights into normal folding mechanisms as well as those that lead to abnormal amyloid fibril conversion. The new insights may lead to the discovery of novel therapeutic targets for neurodegenerative diseases. Intriguingly, certa...

Scientists identify prion's infectious secret

Researchers have known for decades that certain neurodegenerative diseases, such as mad cow disease or its human equivalent, Cruetzfeldt-Jakob disease, result from a kind of infectious protein called a prion. Remarkably, in recent years researchers also have discovered non-pathogenic prions that play beneficial roles in biology, and prions even may act as essential elements in learning and memory...

Vaccine prevents prion disease in mice

An oral vaccine can prevent mice from developing a brain disease similar to mad cow disease, according to research that will be presented at the American Academy of Neurology's 59th Annual Meeting in Boston, April 28 ?May 5, 2007. Prion diseases, which include scrapie, mad cow disease, and chronic wasting disease, are fatal and there is no treatment or cure. The disease spreads when an an...