ScienceDaily: Mad Cow Disease News

Putting the brakes on Parkinson's

Mon, 22 Apr 2013 11:11:11 EDT

The earliest signs of Parkinson's disease can be deceptively mild. The first thing that movie star Michael J. Fox noticed was twitching of the little finger of his left hand. For years, he made light of the apparently harmless tic. But such tremors typically spread, while muscles stiffen up and directed movements take longer to carry out. Research groups have developed a chemical compound that slows down the onset and progression of Parkinson's disease in mice. The scientists hope that this approach will give them a way to treat the cause of Parkinson's and so arrest its progress.

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.

Rapid hearing loss may be a symptom of rare Creutzfeldt-Jakob Disease

Thu, 14 Mar 2013 08:55:55 EDT

Rapid hearing loss in both ears may be a symptom of the rare but always-fatal Creutzfeldt-Jakob Disease and should be considered a reason for clinicians to test for the disorder.

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.

Adding to the list of disease-causing proteins in brain disorders

Sun, 03 Mar 2013 15:49:49 EST

Researchers have found new candidate disease proteins for neurodegenerative disorders. They describe mutations in prion-like segments of two RNA-binding proteins are associated with a rare inherited degeneration disorder affecting muscle, brain, motor neurons and bone (called multisystem proteinopathy) and one case of the familial form of amyotrophic lateral sclerosis.

Molecular system that could help develop potential treatments for conditions such as Alzheimer's disease identified

Thu, 21 Feb 2013 08:47:47 EST

Scientists have identified the molecular system that contributes to the harmful inflammatory reaction in the brain during neurodegenerative diseases. An important aspect of chronic neurodegenerative diseases, such as Alzheimer’s, Parkinson’s, Huntington’s or prion disease, is the generation of an innate inflammatory reaction within the brain. Results from the study open new avenues for the regulation of the inflammatory reaction and provide new insights into the understanding of the biology of microglial cells, which play a leading role in the development and maintenance of this reaction.

The good side of the prion: A molecule that is not only dangerous, but can help the brain grow

Thu, 14 Feb 2013 07:54:54 EST

A few years ago it was found that certain proteins, called prions, when defective are dangerous, as they are involved in neurodegenerative syndromes such as Creutzfeldt-Jakob disease and Alzheimer's disease. But now research is showing their good side, too: when performing well, prions may be crucial in the development of the brain during childhood, as observed by a study carried out by a team of neuroscientists in Italy.

Green tea and red wine extracts interrupt Alzheimer's disease pathway in cells

Tue, 05 Feb 2013 20:02:02 EST

Natural chemicals found in green tea and red wine may disrupt a key step of the Alzheimer's disease pathway, according to new research.

Study confirms no transmission of Alzheimer's proteins between humans

Mon, 04 Feb 2013 18:45:45 EST

A new study found no evidence to support concerns that abnormal neurodegenerative disease proteins are "infectious" or transmitted from animals to humans or from one person to another.

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.

Prion protein hints at role in aiding learning and memory

Tue, 16 Oct 2012 12:56:56 EDT

Scientists have found that the protein called prion helps our brains to absorb zinc, which is believed to be crucial to our ability to learn and the well-being of our memory.

Test can help make diagnosis of Creutzfeldt-Jakob disease

Wed, 19 Sep 2012 19:01:01 EDT

A new guideline may help doctors in making the diagnosis of Creutzfeldt-Jakob disease.

'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.

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 MRI technique may predict progress of dementias

Tue, 10 Apr 2012 21:09:09 EDT

A new technique for analyzing brain images offers the possibility of using magnetic resonance imaging to predict the rate of progression and physical path of many degenerative brain diseases, report scientists.

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.

Alzheimer's might be transmissible in similar way as infectious prion diseases, research suggests

Tue, 04 Oct 2011 11:37:37 EDT

The brain damage that characterizes Alzheimer's disease may originate in a form similar to that of infectious prion diseases such as bovine spongiform encephalopathy (mad cow) and Creutzfeldt-Jakob, according to newly published research.

Screen finds an antidepressant and other drugs that might work against brain-wasting prion diseases

Wed, 14 Sep 2011 11:58:58 EDT

In a new study, researchers report that they have found several chemical compounds, including an antidepressant, that have powerful effects against brain-destroying prion infections in mice, opening the door to potential treatments for human prion diseases.

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.

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.

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.

Creutzfeldt-Jakob disease: Coroners wrong to say no to post-mortem tissue collection, UK academics argue

Mon, 09 May 2011 06:57:57 EDT

The creation of a post-mortem tissue archive for a study of the human form of mad cow disease failed because of a 'misguided' refusal by coroners to participate, academics in the UK argue in a new article.

Needle-in-a-haystack search identifies potential brain disease drug

Wed, 23 Feb 2011 12:24:24 EST

Scientists who examined more than 10,000 chemical compounds during the last year in search of potential new drugs for a group of untreatable brain diseases, are reporting that one substance shows unusual promise. The early positive signs for so-called prion diseases come from research in laboratory mice and cell cultures.

How disordered proteins spread from cell to cell, potentially spreading disease

Fri, 18 Feb 2011 16:52:52 EST

Misfolded proteins can get into cells and form large aggregates by recruiting normal proteins. These aggregates are associated with neurodegenerative diseases. A new study finds that the protein linked to Huntington's can spread from one cell to another. The research may explain how these diseases spread through our brains, an understanding that might lead to the development of drugs to target the misfolded proteins.

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.

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.

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.

Peripheral induction of Alzheimer's-like brain pathology in mice

Thu, 21 Oct 2010 14:14:14 EDT

Pathological protein deposits linked to Alzheimer's disease and cerebral amyloid angiopathy can be triggered not only by the administration of pathogenic misfolded protein fragments directly into the brain but also by peripheral administration outside the brain, according to a new study.

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.

Infectious prions can arise spontaneously in normal brain tissue, study shows

Mon, 26 Jul 2010 16:21:21 EDT

In a startling new discovery, scientists have shown for the first time that abnormal prions, bits of infectious protein devoid of DNA or RNA that can cause fatal neurodegenerative disease, can suddenly erupt from healthy brain tissue.

Surgery linked to Creutzfeldt-Jakob disease, according to Spanish study

Thu, 08 Jul 2010 07:15:15 EDT

A new study spearheaded by Spanish scientists demonstrates a causal relationship between the onset of Creutzfeldt-Jakob disease (CJD), caused by a protein called a prion, and general surgery.

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.

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.

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.

Prion leaves lasting mark on memory

Thu, 04 Feb 2010 14:44:44 EST

Prions are a special class of proteins best known as the source for mad cow and other neurodegenerative diseases. Despite this negative reputation, a prion may also have important and very positive roles in brain function. The researchers suggest that a prion-like protein may participate in memory in higher eukaryotes, from sea slugs on up.

Social TV viewing is disappearing

Mon, 18 Jan 2010 11:17:17 EST

We are watching television together less and less often. "We are becoming more and more individualistic also in our choice of TV programs," according to new research.

Brain disease 'resistance gene' evolves in Papua New Guinea community; could offer insights into CJD

Fri, 20 Nov 2009 09:19:19 EST

A community in Papua New Guinea that suffered a major epidemic of a CJD-like fatal brain disease called kuru has developed strong genetic resistance to the disease, according to new research by scientists in the UK.

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.

Cell study explains why younger people more at risk of variant Creutzfeldt-Jakob disease (vCJD)

Wed, 14 Oct 2009 10:20:20 EDT

Specific cells within the immune system could help explain why younger people are more susceptible to variant Creutzfeldt-Jakob disease -- a rare, degenerative, fatal brain disorder. Patients diagnosed with variant CJD are, on average, 28 years old but it has been unclear why older people are not as affected by the disease.

Impaired Transport In Neurons Triggers Prion Disease

Fri, 21 Aug 2009 13:51:51 EDT

A new study shows that nervous system integrity and axonal properties may play a key role in prion diseases. The findings expand our understanding of the development of prion disease and suggest novel targets for therapeutic and diagnostic approaches in its early stages.

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.

How Prion Protein Damages Brain Cells: Findings Could Advance Understanding Of Mad Cow Disease, Related Disorders

Wed, 10 Jun 2009 12:44:44 EDT

Scientists have gained a major insight into how the rogue protein responsible for mad cow disease and related neurological illnesses destroys healthy brain tissue.

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.

Antibody Key To Treating Variant Creutzfeldt-Jakob Disease (vCJD)

Wed, 04 Mar 2009 10:42:42 EST

Scientists have determined the atomic structure of the "binding" between a brain protein and an antibody that could be key to treating patients with diseases such as variant CJD.

New Piece In Alzheimer's Puzzle

Wed, 25 Feb 2009 13:22:22 EST

Researchers have filled in a missing gap on the molecular road map of Alzheimer's disease. They report that cellular prion proteins trigger the process by which amyloid-beta peptides block brain function in Alzheimer's patients.

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.

Tracking Down The Cause Of Mad Cow Disease: First Synthetic Prion Protein With An Anchor

Wed, 08 Oct 2008 11:34:34 EDT

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.

Protective Pathway In Stressed Cells Not So Helpful When It Comes To Prions

Mon, 15 Sep 2008 12:12:12 EDT

Scientists have discovered that an important cellular quality control mechanism may actually be toxic to some brain cells during prion infection. The research proposes a new general mechanism of cellular dysfunction that can contribute to the devastating and widespread neuronal death characteristic of slowly progressing neurodegenerative diseases.

Variant Of Mad Cow Disease May Be Transmitted By Blood Transfusions, According To Animal Study

Thu, 28 Aug 2008 13:59:59 EDT

Blood transfusions are a valuable treatment mechanism in modern medicine, but can come with the risk of donor disease transmission. Researchers are continually studying the biology of blood products to understand how certain diseases are transmitted in an effort to reduce this risk during blood transfusions.

How Small Molecule Can Take Apart Alzheimer's Disease Protein Fibers

Fri, 16 May 2008 09:44:44 EDT

Researchers have shown, in unprecedented detail, how a small molecule is able to selectively take apart abnormally folded protein fibers connected to Alzheimer's disease and prion diseases. Finding a way to dismantle misfolded proteins has implications for new treatments for a host of neurodegenerative diseases.

First Atomic-level Look At A Protein That Causes Brain Disease

Tue, 22 Apr 2008 15:28:28 EDT

For the first time, researchers have peered deeply at the atomic level into the protein that causes hereditary cerebral amyloid angiopathy -- a disease thought to cause stroke and dementia. The study pinpointed a tiny portion of the protein molecule that is key to the formation of plaques in blood vessels in the brain.

Prions Link Cholesterol To Neurodegeneration

Mon, 11 Feb 2008 19:52:52 EST

Prion infection of neurons increases the free cholesterol content in cell membranes. A new study suggests that disturbances in membrane cholesterol may be the mechanism by which prions cause neurodegeneration and could point to a role for cholesterol in other neurodegenerative diseases.

Chemists Characterize Alzheimer's Neurotoxin Structure

Mon, 03 Dec 2007 13:57:57 EST

Chemists have characterized the molecular structure of the intermediate stage of plaque-forming amyloid fibrils, believed to cause Alzheimer's disease. The finding may lead to new drug targets for this and other amyloid diseases, such as Parkinson's and Creutzfeldt-Jakob.

Prion Fingerprints Detected With Glowing Molecule

Wed, 21 Nov 2007 22:17:17 EST

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.