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Is there more to prion protein than mad cow disease?
September 30, 2008Prion 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. Publishing their findings in the open access journal BMC Veterinary Research, scientists have detected changes in the production and accumulation of the prion protein in the brains of cattle with a rare neurodegenerative disorder.
Martin Jeffrey of the Veterinary Laboratories Agency led a research team that tested 15 brains of cattle with idiopathic brainstem neuronal chromatolysis and hippocampal sclerosis (IBNC). They are the first group to show that the brains of animals with this disease accumulate prion protein (PrP), the protein that misfolds to cause BSE and which, when transmitted to humans through the food chain, can cause the deadly Variant Creutzfeldt-Jakob disease
IBNC is a rare neurological disease of adult cattle. It was first characterised in 1988 following laboratory tests from cattle suspected of having BSE. Although IBNC has some clinical similarities to BSE, the brains of affected cattle do not have the neuronal vacuolation (lesions) typical of BSE.
Further laboratory tests suggest that the misfolded form of PrP, which accumulates in the brains of BSE cases, is not present in IBNC cases. Commercial BSE testing kits did not detect the telltale, BSE-inducing form of PrP either. However, the presence of increased levels of PrP was detected.
"We've shown for the first time that prion protein is somehow involved in IBNC," says Jeffrey, "In this disease, there is an association with abnormally high levels of a prion protein in the brain but clearly this PrP is in a different form to that involved in BSE and CJD. This may have implications for diagnosis and recognition of typical forms of BSE as well as the related diseases in sheep, deer and in man.
BioMed Central
Further laboratory tests suggest that the misfolded form of PrP, which accumulates in the brains of BSE cases, is not present in IBNC cases. Commercial BSE testing kits did not detect the telltale, BSE-inducing form of PrP either. However, the presence of increased levels of PrP was detected.
"We've shown for the first time that prion protein is somehow involved in IBNC," says Jeffrey, "In this disease, there is an association with abnormally high levels of a prion protein in the brain but clearly this PrP is in a different form to that involved in BSE and CJD. This may have implications for diagnosis and recognition of typical forms of BSE as well as the related diseases in sheep, deer and in man.
BioMed Central
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Prion Protein Current Events and Prion Protein News RSSA Penny for Your Prions
North Carolina State University 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.
Scripps Florida scientists devise accelerated method to determine infectious prion strainsScripps Florida scientists devise accelerated method to determine infectious prion strains
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.
Prevalence of variant CJD agent in Britain remains uncertain
First results from a large tissue survey in Britain of the agent that causes variant Creutzfeldt-Jakob disease (vCJD) are unable so far to establish that the prevalence is lower than that given by previous estimates, concludes a study published on bmj.com today.
Iron is involved in prion disease-associated neuronal demise
Imbalance of iron homeostasis is a common feature of prion disease-affected human, mouse, and hamster brains, according to a new study by Dr. Neena Singh and colleagues at Case Western Reserve University School of Medicine, alongside collaborators from Creighton University.
Antibody key to treating variant CJD, scientists find
Scientists at the University of Liverpool 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.
Mutant proteins result in infectious prion disease in mice
A worldwide group of scientists has 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.
K-State professor's USDA research shows mad cow disease also caused by genetic mutation
New findings about the causes of mad cow disease show that sometimes it may be genetic.
How small molecule can take apart Alzheimer's disease protein fibers
Researchers from the University of Pennsylvania School of Medicine 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.
Europe develops new technologies to boost health of livestock
A range of new technologies including genetic modification (GM) and RNA Interference are being deployed to improve the health of farm animals in a series of European and global initiatives. The ground was laid for a European platform to develop new treatments that exploit these technologies at a recent workshop organised by the European Science Foundation (ESF).
New insights into the diversity of Creutzfeldt-Jakob disease agents
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Amyloid, Prions, and Other Protein Aggregates, Part B, Volume 412 by Academic Press The ability of polypeptides to form alternatively folded, polymeric structures such as amyloids and related aggregates is being increasingly recognized as a major new frontier in protein research. This new volume of Methods in Enzymology along with Part C (volume 413) on Amyloid, Prions and other Protein Aggregates continue in the tradition of the first volume (309) in containing detailed protocols and methodological insights, provided by leaders in the field, into the latest methods for investigating the structures, mechanisms of formation, and biological activities of this important class of protein assemblies. * Presents detailed protocols * Includes troubleshooting tips * Provides coverage on structural biology, computational... |
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Amyloid, Prions, and Other Protein Aggregates, Part B, Volume 412 (Methods in Enzymology) by Ronald Wetzel (Editor), Indu Kheterpal (Editor) The ability of polypeptides to form alternatively folded, polymeric structures such as amyloids and related aggregates is being increasingly recognized as a major new frontier in protein research. This new volume of Methods in Enzymology along with Part C (volume 413) on Amyloid, Prions and other Protein Aggregates continue in the tradition of the first volume (309) in containing detailed protocols and methodological insights, provided by leaders in the field, into the latest methods for investigating the structures, mechanisms of formation, and biological activities of this important class of protein assemblies. * Presents detailed protocols * Includes troubleshooting tips * Provides coverage on structural biology, computational... |
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Prions: Concept, Biology, and Applications in Biotechnology by Taylor & Francis This volume offers a well-organized overview of prion-related diseases. It reviews the symptoms, epidemiology, and neuropathology of the disease, and focuses on the idea that TSEs result from a novel mechanism involving transmission by replication of the misfolding of a single protein. The author, a renowned innovator in the area of neurodegenerative diseases, examines the structure, conversion, and mechanism of prion propagation and details its cellular biology. He also looks at other diseases that display folding aberrations, considers how common such aberrations are, and speculates on the impact of prions on broader areas of biology, public health, and biotechnological strategies. |
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Amyloids, Prions and Other Protein Aggregates (Methods in Enzymology, Volume 309) by John N. Abelson (Editor), Melvin I. Simon (Editor), Ronald Wetzel (Editor) This volume includes a core of methodologies to attack the unique experimental problems presented by protein misassembly. Emphasis is on human biology applications, the area in which there is the most interest, in which most of the work has already been done, and in which there is the best evidence for the structural sophisitication of the protein aggregates. The critically acclaimed laboratory standard for more than forty years, Methods in Enzymology is one of the most highly respected publications in the field of biochemistry. Since 1955, each volume has been eagerly awaited, frequently consulted, and praised by researchers and reviewers alike. Now with more than 300 volumes (all of them still in print), the series contains much material still relevant today--truly an essential... |
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The Pathological Protein: Mad Cow, Chronic Wasting, and Other Deadly Prion Diseases by Springer In 1996, British doctors were horrified to discover that mad cow disease (BSE), an affliction that had been plaguing British cattle for ten years, had jumped the species barrier and was appearing in humans as variant Creutzfeldt-Jakob disease (vCJD). Not unlike the mad cows, victims of vCJD suffer from a degenerative neurological disease that peppers the brain with microscopic holes, causing dementia, loss of motor control, and certain death. What alarms researchers and public health officials worldwide is that the incubation period for vCJD may be as long as 10 or even 15 years, and during this period those infected are symptom-free. And because the disease is so far undetectable except by autopsy, there is no way of knowing with certainty how many people have already been infected. In... |
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Fatal Protein: The Story of CJD, BSE, and Other Prion Diseases by Rosalind M. Ridley (Author), Harry F. Baker (Author) This book presents a clear and authoritative survey of the prion diseases, designed mainly for the non-specialist. It deals with the history of scrapie in sheep, the spread of kuru through cannibalism in Papua New Guinea, and the BSE epidemic in Britain--"mad cow disease"--which claimed over 170,000 cattle and threatened the rest of Europe. The authors present a detailed description of the human prion diseases, and examine the evidence that the new variant of the Creutzfeld-Jakob disease results from eating infected by BSE. At the heart of this book lies a discussion of the still controversial idea that the infectious agent in prion diseases is a normal brain protein which takes on abnormal, mutant shape. This is the so-called "prion theory". The potential of similar processes underlying... |
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