Latest PRNP Stories
Prions are a special class of proteins best known as the source for mad cow and other neurodegenerative diseases. Despite this negative reputation, according to a new report in the February 5th issue of the journal Cell, a Cell Press publication, 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."The persistence of memory is a fundamental...
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."...
Research may point to more effective therapeutic targets for deadly prion diseasesScientists from The Scripps Research Institute 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.The study from Scripps Florida in Jupiter shows that prions can develop large numbers of mutations at the protein level and, through natural selection, these mutations can eventually bring...
A new treatment route for bovine spongiform encephalopathy (BSE) and its human form Creutzfeldt Jakob disease (CJD) could be a step closer based on new results from scientists at the University of Leeds. The team has found that a protein called Glypican-1 plays a key role in the development of BSE. Details are published November 20 in the open-access journal PLoS Pathogens.BSE, commonly known as mad cow disease, is known to be caused by an infectious and abnormal form of the prion protein...
A University of Toronto-led team of scientists says it has determined the evolutionary origin of the prion gene. The researchers said the diseased prion proteins are responsible for the fatal neurodegenerative Creutzfeldt-Jakob disease in humans, bovine spongiform encephalopathy, also known as mad cow disease, as well as scrapie and chronic wasting disease in livestock. The scientists said their findings hold promise for efforts to reveal the physiological function of members of the prion...
A University of Toronto-led team has 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 (BSE), also known as "mad cow disease."Diseased prion proteins are responsible for the fatal neurodegenerative Creutzfeldt-Jakob disease (CJD) in humans, and BSE, scrapie and chronic wasting disease (CWD) in livestock. Overall, this work holds promise for efforts to reveal the...
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. 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.Prion proteins, or PrPs, are commonly found in brain...
U.S. medical scientists say they have determined how prion proteins responsible for mad cow disease and related illnesses destroy healthy brain tissue. The National Institutes of Health researchers said their findings will help in developing potential treatments for prion diseases or, perhaps, ways to prevent them. The research, conducted at the Eunice Kennedy Shriver National Institute of Child Health and Human Development, determined the proteins responsible for the disorders, known as...
Development may help shed light on disease in animals and protect human populationsCurrent 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.Now, a group of scientists from The Scripps Research Institute's Florida campus have developed a new method that cuts this critical time lag by...
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.Researchers at the University of California, Santa Cruz, discovered a striking relationship between changes in the copper-binding properties of abnormal prion proteins and the clinical features of prion disease in patients with certain rare, genetic mutations. They described their findings in a paper...
