ScienceDaily: Prion News

Potential therapy for human prion disease

Wed, 03 Apr 2013 15:43:43 EDT

Scientists have for the first time identified a pair of drugs already approved for human use that show anti-prion activity and, for one of them, great promise in treating rare and universally fatal disorders, such as Creutzfeldt-Jakob disease, caused by misfolded proteins called prions.

Protective prion keeps yeast cells from going it alone

Thu, 28 Mar 2013 14:21:21 EDT

A team of scientists has added markedly to the job description of prions as agents of change, identifying a prion capable of triggering a transition in yeast from its conventional single-celled form to a cooperative, multicellular structure. This change, which appears to improve yeast's chances for survival in the face of hostile environmental conditions, is an epigenetic phenomenon -- a heritable alteration brought about without any change to the organism’s underlying genome.

Normal prion protein regulates iron metabolism

Wed, 13 Mar 2013 13:19:19 EDT

An iron imbalance caused by prion proteins collecting in the brain is a likely cause of cell death in Creutzfeldt-Jakob disease, researchers have found. The breakthrough follows discoveries that certain proteins found in the brains of Alzheimer's and Parkinson's patients also regulate iron.

Discovery may explain how prion diseases spread between different types of animals

Mon, 11 Mar 2013 12:42:42 EDT

Medical researchers have made a discovery that may explain how prion diseases, like chronic wasting disease and mad cow disease, adapt in order to spread between various types of animals.

Brain inflammation likely key initiator to Prion and Parkinson's disease

Thu, 29 Nov 2012 09:33:33 EST

Researchers have shown that neuro-inflammation plays a crucial role in initiating prion disease.

Crows don't digest prions, may transport them to other locations

Wed, 17 Oct 2012 18:12:12 EDT

Crows fed on prion-infected brains from mice can transmit these infectious agents in their feces and may play a role in the geographic spread of diseases caused by prions, such as chronic wasting disease or scrapie.

'Mad Cow' blood test now on the horizon

Wed, 12 Sep 2012 09:38:38 EDT

A simple blood test for Creutzfeldt-Jakob Disease and Mad Cow disease is a step closer, following a breakthrough by medical researchers in Australia.

Copper facilitates prion disease, scientists show

Thu, 09 Aug 2012 19:07:07 EDT

Many of us are familiar with prion disease from its most startling and unusual incarnations —- the outbreaks of “mad cow” disease (bovine spongiform encephalopathy) that created a crisis in the global beef industry. Or the strange story of Kuru, a fatal illness affecting a tribe in Papua New Guinea known for its cannibalism. Both are forms of prion disease, caused by the abnormal folding of a protein and resulting in progressive neurodegeneration and death.

New compounds inhibit prion infection

Mon, 23 Jul 2012 13:48:48 EDT

Researchers have identified a new class of compounds that inhibit the spread of prions, misfolded proteins in the brain that trigger lethal neurodegenerative diseases in humans and animals. Scientists have now developed compounds that clear prions from infected cells derived from the brain.

Prions in the brain eliminated by homing molecules

Tue, 24 Apr 2012 09:57:57 EDT

Toxic prions in the brain can be detected with self-illuminating polymers. The originators, at Linköping University in Sweden, has now shown that the same molecules can also render the prions harmless, and potentially cure fatal nerve-destroying illnesses.

New yeast prion helps cells survive

Mon, 23 Apr 2012 10:50:50 EDT

One of the greatest mysterious in cellular biology has been given a new twist. Researchers now show that prions, proteins that transmit heritable information without DNA or RNA, can contribute to drug resistance and cellular adaptation. Their discovery of a yeast prion with these properties demonstrates the active role of the prion conversion in cellular fitness adaptation, providing new insights into the potentially broader function of prions in living organisms.

Most lethal known species of prion protein identified

Thu, 09 Feb 2012 15:28:28 EST

Scientists have identified a single prion protein that causes neuronal death similar to that seen in "mad cow" disease, but is at least 10 times more lethal than larger prion species.

New findings about the prion protein and its interaction with the immune system

Thu, 29 Dec 2011 09:16:16 EST

Scrapie is a neurodegenerative disease which can function as a model for other diseases caused by an accumulation of proteins resulting in tissue malformations (proteinpathies), such as Alzheimer's and Parkinson's disease. Many questions regarding these diseases still remain unanswered. A new study has uncovered a number of factors relating to the uptake of the prion protein (PrPSc) associated with the development of this disease and how this protein interacts with the immune cells in the intestines.

Cellular stress can induce yeast to promote prion formation

Fri, 22 Jul 2011 21:34:34 EDT

Biochemists have identified a yeast protein called Lsb2 that can promote spontaneous prion formation. Prions can cause neurodegenerative disorders, such as mad cow/Creutzfeld-Jakob disease, in humans and animals.

Tracking down BSE and Creutzfeldt-Jakob disease

Thu, 30 Jun 2011 09:17:17 EDT

Researchers have identified an altered expression of endogenous retroviruses in BSE-infected macaques.

Two gene classes linked to new prion formation

Thu, 26 May 2011 12:29:29 EDT

Researchers have discovered two classes of yeast genes that may hold clues as to why proteins take on the misfolded prion form, a condition associated with several neurodegenerative diseases, such as "mad cow."

Potential human exposure to prion diseases assessed

Mon, 23 May 2011 07:53:53 EDT

Researchers have examined the potential for human exposure to prion diseases, looking at hunting, venison consumption, and travel to areas in which prion diseases have been reported in animals. Three prion diseases in particular -- bovine spongiform encephalopathy, variant Creutzfeldt-Jakob disease and chronic wasting disease -- were specified in the investigation.

Lichens may aid in combating deadly chronic wasting disease in wildlife

Wed, 18 May 2011 14:17:17 EDT

Certain lichens can break down the infectious proteins responsible for chronic wasting disease (CWD), a troubling neurological disease fatal to wild deer and elk and spreading throughout the United States and Canada.

Fast, sensitive blood test for human prion disease

Mon, 09 May 2011 17:18:18 EDT

Scientists have developed a method -- 10,000 times more sensitive than other methods -- to detect variant Creutzfeldt-Jacob disease (vCJD) in blood plasma. vCJD is a type of prion disease in humans that leads to brain damage and death. The researchers also used the test to rapidly detect scrapie, a prion disease of sheep, in infected hamsters, some pre-symptomatic.

New research focuses on prion diseases

Mon, 14 Mar 2011 16:36:36 EDT

New research may shed light on possible treatments for prion diseases.

Unraveling how prion proteins move along axons in the brain

Thu, 17 Feb 2011 12:51:51 EST

Researchers have identified the motors that move non-infectious prion proteins -- found within many mammalian cells -- up and down long, neuronal transport pathways. Identifying normal movement mechanisms of PrPC may help researchers understand the spread of infectious prions within and between neurons to reach the brain, and aid in development of therapies to halt the transport.

Prion disease spreads in sheep via mother's milk

Wed, 19 Jan 2011 19:13:13 EST

Transmission of prion brain diseases such as bovine spongiform enecephalopathy (BSE) -- also known as mad cow disease -- and human variant Creutzfeldt-Jakob disease (vCJD) is generally attributed to the consumption of the brain or organ meat of infected animals but new research demonstrates lambs exposed to milk from prion-infected sheep with inflamed mammary glands can develop prion disease as well. The research has major implications for human and livestock health.

Airborne pathogens can induce mad cow disease, new findings suggest

Thu, 13 Jan 2011 21:30:30 EST

Airborne prions are also infectious and can induce mad cow disease or Creutzfeldt-Jakob disorder, new findings suggest. Researchers recommend precautionary measures for scientific labs, slaughterhouses and animal feed plants.

New drug target for prion diseases, 'mad cow'

Mon, 10 Jan 2011 10:38:38 EST

Scientists have discovered that plasminogen, a protein used by the body to break up blood clots, speeds up the progress of prion diseases such as mad cow disease.

Prions mutate and adapt to host environment

Fri, 17 Dec 2010 08:32:32 EST

Scientists have shown that prions, bits of infectious protein that can cause fatal neurodegenerative disease such as bovine spongiform encephalopathy (BSE) or "mad cow disease," have the ability to adapt to survive in a new host environment.

Early detection is possible for prion diseases, study suggests

Thu, 02 Dec 2010 18:11:11 EST

A fast test to diagnose fatal brain conditions such as mad cow disease in cattle and Creutzfeldt-Jakob disease in humans could be on the horizon, according to a new study. Researchers have developed a highly sensitive and rapid new method to detect and measure infectious agents called prions that cause these diseases.

New prion discovery reveals drug target for mad cow disease and related illnesses

Wed, 01 Dec 2010 10:26:26 EST

In a new study, scientists have found that a protein our body uses to break up blood clots speeds up the progress of prion diseases. This substance, called plasminogen, is a new drug target for prion diseases in both humans and animals.

Size of protein aggregates, not abundance, drives spread of prion-based disease

Thu, 28 Oct 2010 14:17:17 EDT

In a study that challenges the conventional wisdom about infections caused by proteins called prions, researchers report that the size of protein structures, rather than their abundance, determines their transmission among cells.

Eye test for neurological diseases in livestock developed

Tue, 19 Oct 2010 15:26:26 EDT

Researchers have discovered that the eyes of sheep infected with scrapie return an intense, almost-white glow when they're hit with blue light. That finding suggests technologies can be developed to quickly and non-invasively test for transmissible spongiform encephalopathies, progressive and fatal neurological diseases such as mad cow disease.

New sporadic prion protein disease: Variably protease-sensitive prionopathy shares genotype characteristics with Creutzfeldt-Jakob

Fri, 13 Aug 2010 11:02:02 EDT

A new sporadic prion protein disease has been discovered. Variably protease-sensitive prionopathy, as it has been named, is the second type of complete sporadic disease to be identified since Creutzfeldt-Jakob disease was reported in the 1920s.

Sequence and structure key to prion disease transmission

Mon, 14 Jun 2010 12:15:15 EDT

Prion diseases are lethal neurodegenerative disorders that include Creutzfeldt-Jakob disease in humans and bovine spongiform encephalopathy (BSE, commonly known as mad cow disease) in cows. New data generated in mice provides greater understanding of the factors that determine how easy it is for prion diseases to be transmitted to a new host species. This information provides new insight into a highly important food safety issue.

Eyes of cattle may become new windows to detect mad cow disease

Wed, 02 Jun 2010 12:11:11 EDT

The eyes may or may not be windows to the soul, as the old adage goes, but scientists are reporting evidence that a peek into the eyes of cattle may become the basis for a long-sought test to detect infection with the agent that causes Mad Cow Disease. That test could help prevent the disease from spreading in the food supply.

New model for chronic wasting disease

Wed, 26 May 2010 10:39:39 EDT

Scientists have generated a mouse model of cervid chronic wasting disease. Chronic wasting disease is a fatal prion-induced disease, similar to mad cow disease, that affects cervids such as deer, elk, and moose. It is a neurodegenerative disease typified by chronic weight-loss leading to death.

New form of prion disease damages brain arteries

Thu, 04 Mar 2010 20:22:22 EST

Scientists investigating how prion diseases destroy the brain have observed a new form of the disease in mice that does not cause the sponge-like brain deterioration typically seen in prion diseases. Instead, it resembles a form of human Alzheimer's disease, cerebral amyloid angiopathy, that damages brain arteries.

Researchers induce a new transmissible prion disease

Fri, 26 Feb 2010 21:14:14 EST

Researchers have conducted a study on prion disease and found that transmissible spongiform encephalopathy (TSE) can be induced without an outside catalyst like a virus.

Prion protein in cell culture

Fri, 05 Feb 2010 11:59:59 EST

The fatal brain disease Creutzfeldt-Jakob in humans, BSE (bovine spongiform encephalopathy) in cattle and scrapie in sheep are so-called prion diseases, whereby one of the body's normal proteins, the prion protein PrPc misfolds into a pathogenic form: PrPSc. In spite of several years of extensive research, little is still known about what actually happens in this process.

Spongiform brain diseases are caused by aberrant protein, new research shows

Thu, 28 Jan 2010 14:21:21 EST

Scientists have determined how a normal protein can be converted into a prion, an infectious agent that causes fatal brain diseases in humans and mammals. The finding, in mice, is expected to advance the understanding of transmissible spongiform encephalopathies, or TSEs, a family of neurodegenerative diseases that include Creutzfeldt-Jakob Disease, kuru and fatal familial insomnia in humans, scrapie in sheep, and bovine spongiform encephalopathy in cattle, also known as "mad cow disease."

'Lifeless' prions capable of evolutionary change and adaptation

Thu, 31 Dec 2009 16:47:47 EST

Scientists have determined for the first time that prions, bits of infectious protein devoid of DNA or RNA that can cause fatal neurodegenerative disease, are capable of Darwinian evolution.

Researchers find new piece of bovine spongiform encephalopathy (BSE) puzzle

Thu, 19 Nov 2009 21:08:08 EST

A new treatment route for bovine spongiform encephalopathy (BSE) and its human form Creutzfeldt Jakob disease could be a step closer based on new results from scientists in the UK. The team has found that a protein called Glypican-1 plays a key role in the development of BSE.

Protein Srebp2 drives cholesterol formation in prion-infected neuronal cells

Wed, 18 Nov 2009 10:14:14 EST

The regulating protein Srebp2 drives cholesterol formation, which prions need for their propagation, in prion-infected neuronal cells. Scientists anticipate new approaches in drug development to combat prion infection, as a result of these new findings

First Direct Information About Prion's Molecular Structure Reported

Mon, 05 Oct 2009 16:13:13 EDT

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.

Evolutionary Origins Of Prion Disease Gene Uncovered

Mon, 28 Sep 2009 13:12:12 EDT

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."

Can Gene Expression Profiling Make It Possible To Predict Deadly Infections In Cattle?

Thu, 10 Sep 2009 19:14:14 EDT

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.

Gene Mutation Alone Causes Transmissible Prion Disease

Wed, 26 Aug 2009 15:25:25 EDT

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.

People Who Eat Deer And Elk With Chronic Wasting Disease May Avoid Infection Because Of Species Barrier, Study in Monkeys Suggests

Thu, 30 Jul 2009 11:11:11 EDT

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.

Mad Cow And Related Diseases: Copper Linked To Normal Functioning Of Prions

Thu, 25 Jun 2009 14:15:15 EDT

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.

Farmed Fish May Pose Risk For Mad Cow Disease

Tue, 16 Jun 2009 08:01:01 EDT

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.

Scientists Devise Accelerated Method To Determine Infectious Prion Strains

Thu, 28 May 2009 20:38:38 EDT

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.

Closing In On Goat Scrapie

Sun, 19 Apr 2009 20:18:18 EDT

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.

Disruption Of Copper Regulation As Key To Prion Diseases

Fri, 17 Apr 2009 08:41:41 EDT

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.

Large Number Of New Prions Discovered: Scientists Redefining What It Means To Be A Prion

Thu, 02 Apr 2009 12:43:43 EDT

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.

Prion Discovery Gives Clue To Control Of Mass Gene Expression

Fri, 13 Mar 2009 17:13:13 EDT

The discovery of a new yeast prion may provide clues on whether prions, like proteins, can affect mass activation of gene expression.

Iron Is Involved In Prion Disease-associated Neuronal Demise

Fri, 13 Mar 2009 14:59:59 EDT

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.

What Drove The Cow Mad? Lessons From A Tiny Fish

Mon, 09 Mar 2009 20:53:53 EDT

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.

Potential Health Risks Associated With Stressed Foodstuffs Such As Foie Gras

Tue, 10 Feb 2009 09:27:27 EST

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.

New Disease, Comparable To BSE, Created In Laboratory Mice

Tue, 27 Jan 2009 21:44:44 EST

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.

Intestinal Lymphatic Tissue Important For The Absorption And Spread Of The Scrapie Prion

Fri, 09 Jan 2009 09:51:51 EST

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.

New Mouse Model Of Prion Disease: Mutant Proteins Result In Infectious Prion Disease In Mice

Fri, 05 Dec 2008 12:29:29 EST

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.

Prion Infectivity Found In White And Brown Fat Tissues Of Mice

Fri, 05 Dec 2008 09:45:45 EST

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.

Mouse Model Of Prion Disease Mimics Diverse Symptoms Of Human Disorder

Wed, 26 Nov 2008 13:33:33 EST

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.