Prions

Prions News -- ScienceDaily Learn all about prions and prion disease. How are prions linked to mad cow disease, TSE, Creutzfeldt-Jacob disease and chronic wasting disease?

  • Chronic wasting disease
    on September 26, 2017 at 2:54 pm

    New research 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. […]

  • New trick up their sleeve
    on January 17, 2017 at 7:12 pm

    Nerve-damaging protein particles called prions have long been known to exist in mammals. Now, in a surprising discovery, investigators report they have found evidence that bacteria can also make prions. Prions—self-propagating clumps of misfolded protein—have been identified as the cause of several rare but universally fatal neurodegenerative conditions, including bovine spongiform encephalopathy, popularly known as mad cow disease. […]

  • Newly discovered infectious prion structure shines light on mad cow disease
    on September 8, 2016 at 7:12 pm

    Groundbreaking research has identified the structure of the infectious prion protein, the cause of 'mad cow disease' or BSE, chronic wasting disease in deer and elk and Creutzfeldt-Jakob disease in humans, which has long remained a mystery. […]

  • Prion-like protein found in plants
    on April 29, 2016 at 1:51 pm

    Scientists have determined that a plant protein involved in the timing of flowering could in fact be a prion. This is the first time that a possible prion has been identified in plants, and it may play a role in a plant's 'memory' of cold exposure during winter. […]

  • New assay offers improved detection of deadly prion diseases
    on April 8, 2016 at 3:23 pm

    Transmissible spongiform encephalopathies (TSEs), or prion diseases, are a family of rare progressive, neurodegenerative illnesses that affect both humans and animals. TSE surveillance is important for public health and food safety because TSEs have the potential of crossing from animals to humans, as seen with the spread of mad cow disease, or bovine spongiform encephalopathy (BSE). A new study describes an advanced assay that offers better sensitivity than currently available tests for detecting a prion disease affecting elk. […]

  • Why it's hard to make a bunny mad
    on August 6, 2015 at 6:44 pm

    Rabbits have long been considered immune to prion disease, but recently scientists have shown that they can -- under certain circumstances -- get transmissible spongiform encephalopathy (or TSE, the scientific term for the fatal brain disease caused by prions). Two studies address what makes rabbits hard to infect with prions and how their resistance can be overcome. […]

  • Prion trials and tribulations: Finding the right tools and experimental models
    on July 2, 2015 at 7:12 pm

    Prions are fascinating, enigmatic, and might teach us not only about rare prion diseases like Creutzfeld-Jakob disease, mad cow disease, or scrapie, but also about other more common neurodgenerative diseases. Two studies report progress with novel tools and paradigms to study prion disease. […]

  • Grass plants can transport infectious prions
    on May 15, 2015 at 7:56 pm

    Grass plants can bind, uptake and transport infectious prions, according to researchers. Prions are the protein-based infectious agents responsible for a group of diseases called transmissible spongiform encephalopathy, which includes bovine spongiform encephalopathy (mad cow disease) in cattle, scrapie in sheep, variant Creutzfeldt-Jakob disease in humans and chronic wasting disease (CWD) in deer, elk and moose. All are fatal brain diseases with incubation periods that last years. […]

  • Small loop in human prion protein prevents chronic wasting disease
    on February 24, 2015 at 1:33 pm

    Chronic wasting disease (CWD) affects North American elk and deer, but has not been observed in humans. Using a mouse model that expresses an altered form of the normal human prion protein, researchers have determined why the human proteins aren’t corrupted when exposed to the elk prions. Their study identifies a small loop in the human prion protein that confers resistance to chronic wasting disease. […]

  • Scrapie could breach the species barrier
    on December 24, 2014 at 3:30 pm

    The pathogens responsible for scrapie in small ruminants (prions) have the potential to convert the human prion protein from a healthy state to a pathological state, researchers have discovered for the first time. In mice models reproducing the human species barrier, this prion induces a disease similar to Creutzfeldt-Jakob disease. These primary results stress the necessity to reassess the transmission of this disease to humans.  &nbs […]

  • First successful vaccination against 'mad cow'-like wasting disease in deer
    on December 21, 2014 at 11:37 pm

    Researchers say that a vaccination they have developed to fight a brain-based, wasting syndrome among deer and other animals may hold promise on two additional fronts: protecting US livestock from contracting the disease, and preventing similar brain infections in humans. […]

  • Infectious prion protein discovered in urine of patients with variant Creutzfeldt-Jakob disease
    on August 7, 2014 at 2:36 pm

    The misfolded and infectious prion protein that is a marker for variant Creutzfeldt-Jakob disease – linked to the consumption of infected cattle meat – has been detected in the urine of patients with the disease. Variant Creutzfeldt-Jakob disease in humans and bovine spongiform encephalopathy in animals – also known as Mad Cow disease – are fatal neurodegenerative disorders. There are currently no noninvasive tools available to diagnose the disease and there are no treatments. […]

  • New test detects toxic prions in blood
    on June 12, 2014 at 9:46 pm

    The first cases of mad cow disease in humans occurred in the late 1990s and are thought to be the consequence of eating contaminated beef products. Several cases of secondary infections caused by transfusions with blood from donors who developed vCJD have been reported, raising concerns about the safety of blood products. A new article describes an assay that can detect prions in blood samples from humans with vCJD and in animals at early stages of the incubation phase. […]

  • What bank voles can teach us about prion disease transmission and neurodegeneration
    on April 4, 2014 at 1:25 am

    Transmission of prions between species is inefficient, and only a small proportion of exposed recipients become sick within their lifetimes. A new study takes a close look at one exception to this rule: bank voles appear to lack a species barrier for prion transmission, and their universal susceptibility turns out to be both informative and useful for the development of strategies to prevent prion transmission. […]

  • Blood test to screen for fatal variant Creutzfeldt-Jakob disease
    on March 4, 2014 at 3:26 pm

    A blood test accurately screened for infection with the agent responsible for variant Creutzfeldt-Jakob disease (vCJD), a fatal neurological disease. vCJD is a fatal degenerative brain disorder thought to be caused by a misfolded protein (prion) in the brain and contracted most commonly through eating infected beef. Up to 3 million cattle in the United Kingdom may have been infected with BSE (bovine spongiform encephalopathy), and establishing accurate prevalence estimates through screening for vCJD infection would guide public health initiatives. […]

  • Connection found in pathogenesis of neurological diseases, HIV
    on February 4, 2014 at 4:18 pm

    A new study published shows similarities in the pathogenesis of prion disease -- misfolded proteins that can lead to neurological diseases -- and the HIV virus. […]

  • The shape of infectious prions
    on January 24, 2014 at 1:26 pm

    Prions are unique infective agents -- unlike viruses, bacteria, fungi and other parasites, prions do not contain either DNA or RNA. Despite their seemingly simple structure, they can propagate their pathological effects like wildfire, by "infecting" normal proteins. PrPSc (the pathological form of the prion protein) can induce normal prion proteins (PrPC) to acquire the wrong conformation and convert into further disease-causing agents. […]

  • Breakthrough in understanding secret life of prion molecules
    on January 16, 2014 at 7:46 pm

    New research has uncovered a quality control mechanism in brain cells that may help keep deadly neurological diseases in check for months or years. […]

  • Targeted culling of deer controls disease with little effect on hunting
    on October 21, 2013 at 5:10 pm

    Chronic wasting disease, the deer-equivalent of mad cow disease, has crept across the US landscape from west to east, and was first detected in the Midwest in 2002. Little is known about its potential to infect humans. Now researchers offer a first look at the long-term effectiveness of the practice of culling deer in areas affected by CWD to keep the disease in check. […]

  • New models advance the study of deadly human prion diseases
    on August 19, 2013 at 8:25 pm

    By directly altering the gene coding for the prion protein (PrP), researchers have created mouse models of two neurodegenerative prion diseases, each of which manifests in different regions of the brain. These new models for fatal familial insomnia (FFI) and Creutzfeldt-Jakob disease (CJD) accurately reflect the distinct patterns of destruction caused by the these diseases in humans. […]

  • Flexible tail of the prion protein poisons brain cells
    on July 31, 2013 at 5:31 pm

    Prion proteins are the infectious pathogens that cause Mad Cow Disease and Creutzfeldt-Jakob disease. They occur when a normal prion protein becomes deformed and clumped. The naturally occurring prion protein is harmless and can be found in most organisms. In humans, it is found in our brain cell membrane. By contrast, the abnormally deformed prion protein is poisonous for the brain cells. Scientists have now discovered that the prion protein has a kind of switch that controls its toxicity. […]

  • The ribosome: New target for antiprion medicines
    on July 2, 2013 at 2:01 pm

    The key to treating neurodegenerative prion diseases such as mad cow disease and Creutzfeldt-Jakob disease may lie in the ribosome, the protein synthesis machinery of the cell. Prion diseases are fatal neurodegenerative diseases caused by misfolding of prion proteins. Examples of prion diseases are scrapie in sheep, mad cow disease and Creutzfeldt-Jakob disease in human. […]

  • Potential therapy for human prion disease
    on April 3, 2013 at 7:43 pm

    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
    on March 28, 2013 at 6:21 pm

    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
    on March 13, 2013 at 5:19 pm

    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
    on March 11, 2013 at 4:42 pm

    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
    on November 29, 2012 at 2:33 pm

    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
    on October 17, 2012 at 10:12 pm

    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
    on September 12, 2012 at 1:38 pm

    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
    on August 9, 2012 at 11:07 pm

    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. […]