Current Prions News and Events

Current Prions News and Events, Prions News Articles.
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Prion diseases: new clues in the structure of prion proteins
A new study carried out by SISSA - Scuola Internazionale Superiore di Studi Avanzati in collaboration with other institutions including Genos Glycoscience. Research Laboratory from Zagreb, Croatia and Elettra Sincrotrone Trieste, provides important information on the differences in structures of the prions, proteins responsible for diseases that at the state of the art are incurable. (2021-02-19)

The long road to dementia
Alzheimer's disease develops over decades. It begins with a fatal chain reaction in which masses of misfolded beta-amyloid proteins are produced that in the end literally flood the brain. Researchers from the Hertie Institute for Clinical Brain Research and the German Center for Neurodegenerative Diseases show in the journal Nature Neuroscience that this chain reaction starts much earlier in mice than commonly assumed. (2020-11-17)

'Rare' brain disorder may not be so rare anymore, trends in japan reveal
Creutzfeldt-Jakob disease (CJD), a fatal neurodegenerative disorder, is an important cause of dementia. However, long-term trends in CJD-associated mortality and incidence rates in Japan have not been fully studied until now. Now, scientists conducted a detailed analysis that uncovered that the CJD-associated death and incidence rates almost doubled from 2005-2014. They predict that the burden of CJD would continue to rise in the current hyper-aging era. These findings can help policymakers plan and strategize effectively. (2020-10-19)

Naturally occurring antibodies against prion proteins found in humans
Antibodies targeting the normal PrP version of the prion protein have been found in humans selected at random with no history of any associated transmissible spongiform encephalopathies. In EMBO Molecular Medicine, researchers at the University of Zurich and Novartis Institutes for BioMedical Research in Switzerland report on active antibodies against PrP at high levels in a small proportion of individuals, 21 of 37,894 hospital patients screened for presence of anti-PrP IgGs, the most common form of immunoglobulin. (2020-08-17)

Researchers identify a protein that may help SARS-CoV-2 spread rapidly through cells
Eric Ross and Sean Cascarina, biochemistry and molecular biology researchers at Colorado State University, have released a research paper identifying a protein encoded by SARS-CoV-2, the virus that causes COVID-19, that may be associated with the quick spread of the virus through cells in the human body. Through powerful application of the foundational sciences and bioinformatic analysis their research highlights key characteristics of the virus that could one day be important in the development of a treatment for COVID-19. (2020-08-12)

Researchers find new potential treatment for prion diseases
A new study in Nucleic Acids Research suggests a possible effective treatment strategy for patients suffering from prion disease. (2020-08-10)

Unraveling one of prion disease's deadly secrets
In a new paper in Nature Structural and Molecular Biology by Tricia Serio, dean of the College of Natural Sciences and professor of biochemistry and molecular biology at UMass Amherst, and others, report an unanticipated role for prion nucleation seeds that enhances their ability to appear and resist curing. (2020-05-05)

How understanding the dynamics of yeast prions can shed light on neurodegenerative diseases
How understanding the dynamics of yeast prions can shed light on neurodegenerative diseases (2020-04-06)

A game-changing test for Prion, Alzheimer's and Parkinson's diseases is on the horizon
A new test agent can easily and efficiently detect the misfolded protein aggregates that cause devastating neurological diseases in blood samples. The technology could lead to early diagnosis of prion diseases, Alzheimer's and Parkinson's for the first time. (2019-11-06)

Household bleach inactivates chronic wasting disease prions
A 5-minute soak in a 40% solution of household bleach decontaminated stainless steel wires coated with chronic wasting disease (CWD) prions, according to a new study published in PLOS One. The scientists used the wires to model knives and saws that hunters and meat processors use when handling deer, elk and moose - all of which are susceptible to CWD. The research was conducted at Rocky Mountain Laboratories, a component of NIAID. (2019-10-04)

Experimental treatment slows prion disease, extends life of mice
Scientists using an experimental treatment have slowed the progression of scrapie, a degenerative central nervous disease caused by prions, in laboratory mice and greatly extended the rodents' lives, according to a new report in JCI Insight. The scientists used antisense oligonucleotides (ASOs), synthetic compounds that inhibit the formation of specific proteins. (2019-08-01)

Mad cow disease: A computational model reveals the mechanism of replication of prions
An article published in the journal PLOS Pathogens reports a realistic computational model for the structure and mechanism of replication of prions, infectious agents responsible for mad cow disease and other neurodegenerative disorders of human and animals. (2019-07-11)

NIAID scientists develop 'mini-brain' model of human prion disease
Scientists have used human skin cells to create what they believe is the first cerebral organoid system, or 'mini-brain,' for studying sporadic Creutzfeldt-Jakob disease (CJD). CJD is a fatal neurodegenerative brain disease of humans believed to be caused by infectious prion protein. The researchers, from NIH's National Institute of Allergy and Infectious Diseases, hope the human organoid model will enable them to evaluate potential CJD therapeutics and provide greater detail about human prion disease subtypes. (2019-06-14)

Breakthrough in chronic wasting disease research reveals distinct deer, elk prion strains
Researchers have developed a new gene-targeted approach to study chronic wasting disease in mice, allowing opportunities for research that has not previously existed. (2019-06-10)

Analyzing a protein from the cerebrospinal fluid will help diagnose patients with prion diseases
Recently, a study published in the Journal of Neurology, Neurosurgery & Psychiatry demonstrates that very high levels of neurogranin in the cerebrospinal fluid can be detected in human patients that suffer from prion diseases. (2019-05-29)

Alzheimer's disease is a 'double-prion disorder,' study shows
Two proteins central to the pathology of Alzheimer's disease act as prions -- misshapen proteins that spread through tissue like an infection by forcing normal proteins to adopt the same misfolded shape -- according to new UC San Francisco research. (2019-05-01)

Retinal prion disease study redefines role for brain cells
National Institutes of Health scientists studying the progression of inherited and infectious eye diseases that can cause blindness have found that microglia, a type of nervous system cell suspected to cause retinal damage, surprisingly had no damaging role during prion disease in mice. In contrast, the study findings indicated that microglia might delay disease progression. (2019-03-27)

Scientists identify gene that keeps PTSD-like behavior at bay in female mice
More than 30 years ago, scientists discovered that mad cow and Creutzfeldt-Jakob diseases are caused by prions. But in recent years, Nobel laureate Eric Kandel, M.D., demonstrated in mice that some prions are beneficial and serve important functions in the brain and body. And today, new Columbia research from Dr. Kandel describes how one such prion-like protein helps the brain keep fearful memories in check. Without it, female mice exhibit the tell-tale signs of post-traumatic stress disorder, or PTSD. (2019-03-12)

New skin test detects prion infection before symptoms appear
Prions can infect both humans and animals, causing Creutzfeldt-Jakob disease (CJD) in humans, mad cow disease in cattle, and chronic wasting disease in elk and deer. The infectious, misfolded protein particles often go undetected as they destroy brain tissue, causing memory loss, mobility issues, and ultimately death. Preclinical detection of prions has proven difficult, but new research suggests skin samples hold early signs of prion disease that precede neurologic symptoms. (2019-01-22)

Amyloid pathology transmission in lab mice and historic medical treatments
A UCL-led study has confirmed that some vials of a hormone used in discontinued medical treatments contained seeds of a protein implicated in Alzheimer's disease, and are able to seed amyloid pathology in mice. (2018-12-13)

Soil compound fights chronic wasting disease
A major compound in soil organic matter degrades chronic wasting disease prions and decreases infectivity in mice, according to a study published Nov. 29 in the open-access journal PLOS Pathogens by Judd Aiken of the University of Alberta, and colleagues. (2018-11-29)

How prions invade the brain
The spread of prions to the brain does not occur by direct transmission across the blood-brain barrier, according to a study published Nov. 29, 2018, in the open-access journal PLOS Pathogens by Annika Keller and Adriano Aguzzi of the University Hospital Zürich, and colleagues. As noted by the authors, insights into how prions enter the brain could lead to the development of effective strategies to prevent neurodegeneration, even after infection outside the nervous system has already taken place. (2018-11-29)

The eyes have it
By the time symptoms of sporadic Creutzfeldt-Jakob disease (sCJD) are typically discovered, death is looming and inevitable. In a new study, researchers at University of California San Diego School of Medicine with colleagues at the National Institutes of Health and UC San Francisco, report finding tell-tale evidence of the condition's infectious agent in the eyes of deceased sCJD patients, making the eye a potential source for both early CJD detection and prevention of disease transmission. (2018-11-20)

Eyes of CJD patients show evidence of prions
NIH scientists and colleagues have found evidence of the infectious agent of sporadic Creutzfeldt-Jakob disease (CJD) in the eyes of deceased CJD patients. The finding suggests that the eye may be a source for early CJD diagnosis and raises questions about the safety of routine eye exams and corneal transplants. Sporadic CJD, a fatal neurodegenerative prion disease of humans, is untreatable and difficult to diagnose. (2018-11-20)

Creutzfeldt-Jakob disease spreads prions throughout the eyes, researchers find
Researchers recently studied the eyes of 11 people with sporadic Creutzfeldt-Jakob disease (sCJD), the most common and well-known prion disorder. This week in mBio, they report finding prion seeds -- the infectious proteins that cause the disease -- spread throughout the eyes of all the patients. (2018-11-20)

BU researchers define possible molecular pathway for neurodegeneration in prion diseases
A new study has shed light on the mechanisms underlying the progression of prion diseases and identified a potential target for treatment. (2018-09-21)

Hsp90: More than just a chaperone
Researchers from the Verstreken lab (VIB-KU Leuven) have identified a completely novel function for Hsp90, one of the most common and most studied proteins in our body. In addition to its well-known role as a protein chaperone, Hsp90 stimulates exosome release. These findings shed new light on treatment strategies for both cancer and neurodegenerative diseases. (2018-09-07)

New assay reveals biophysical properties that allow certain proteins to infect others
Scientists at the Stowers Institute for Medical Research have identified a physical basis for the spread of corrupted proteins known as prions inside cells. Their research findings are reported in the July 5, 2018, issue of the scientific journal Molecular Cell. (2018-07-05)

Cross-species prion adaptation depends on prion replication environment
A hamster prion that replicated under conditions of low RNA levels in mouse brain material resulted in altered disease features when readapted and transmitted back to hamsters, according to new research presented in PLOS Pathogens by Elizaveta Katorcha of the University of Maryland School of Medicine, Baltimore, and colleagues. (2018-06-21)

CWRU School of Medicine researchers create first artificial human prion
Case Western Reserve University School of Medicine researchers have synthesized the first artificial human prion, a dramatic development in efforts to combat a devastating form of brain disease that has so far eluded treatment and a cure. The new findings are published in Nature Communications. (2018-06-05)

Microglia are key defenders against prion diseases
Prion diseases are slow degenerative brain diseases that occur in people and various other mammals. No vaccines or treatments are available, and these diseases are almost always fatal. Scientists have found little evidence of a protective immune response to prion infections. Further, microglia--brain cells usually involved in the first level of host defense against infections of the brain--have been thought to worsen these diseases by secreting toxic molecules that can damage nerve cells. (2018-05-17)

CWD prions discovered in Wisconsin soils for the first time
New research out of the University of Wisconsin-Madison has, for the first time, detected prions responsible for chronic wasting disease (CWD) in samples taken from sites where deer congregate. (2018-05-03)

Soil characteristics may be related to chronic wasting disease persistence, study finds
Deer infected with chronic wasting disease are doomed to a slow and certain death, eventually wasting away as they lose the ability to eat and drink. There is no cure and no vaccine, and the number of infected deer continues to rise every year. But University of Illinois scientists recently published a new study that could help explain the movement of the disease across the landscape. (2018-02-05)

The bright side of an infectious protein
Prions are self-propagating protein aggregates that can be transmitted between cells. The aggregates are associated with human diseases. 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. (2018-01-09)

Researchers find infectious prions in Creutzfeldt-Jakob disease patient skin
In a Science Translational Medicine study published today, Case Western Reserve University School of Medicine researchers found that CJD patients also harbor infectious prions in their skin, albeit at lower levels. (2017-11-22)

Getting under the skin of prion disorders
Infectious prion proteins -- the causative agents of the fatal neurodegenerative disorder Creutzfeldt-Jakob disease -- can be detected in the skin of afflicted individuals, researchers now report. (2017-11-22)

Brain cell advance brings fresh hope for CJD therapies
Scientists at the University of Edinburgh have developed a new system to study Creutzfeldt-Jakob disease in the laboratory, paving the way for research to find treatments for the fatal brain disorder. (2017-11-20)

BU researchers create tool to measure, control protein aggregation
In the cover article in the current issue of Cell, BU Biomedical Engineer Ahmad S. Khalil along with colleagues from MIT and the Whitehead Institute for Biomedical Research, among others, describe the synthetic genetic tool they built to quantitatively sense, measure and manipulate protein aggregation in live cells. This may open the door to greater understanding and treatment of a range of maladies from Alzheimer's to type II diabetes. (2017-10-20)

Chronic wasting disease
Research published in the International Journal of Global Environmental Issues, summarizes the efforts in disease surveillance and risk management of chronic wasting disease (CWD) in deer and shows that past management strategies such as selective culling, herd reduction, and hunter surveillance have had only limited effectiveness. The summary points towards new advice for optimal, cost-effective strategies in aggressive disease control. (2017-09-26)

Parkinson's disease and prion diseases: Discovery of a molecular link
Parkinson's disease and prion diseases are very different from each other as regards both origins and course. Nonetheless, a research group of SISSA's has discovered an unexpected and important link between the two pathologies. According to the study recently published in the journal Scientific Reports, the link is given by the complex interaction between two different proteins present in our nerve cells: the so-called α-synuclein, in its aggregated form, and the prion protein PrPC. (2017-09-14)

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