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Silencing the cause of mad cow disease
December 04, 2006BSE (more commonly known as mad cow disease) and CJD, which is a related disease in humans that can occur spontaneously, be inherited, or be acquired (in some cases probably from cows with BSE), are fatal neurodegenerative diseases. It is thought that these diseases are caused by accumulation in the brain of an abnormally folded version (PrPsc) of a natural protein (PrPc). There are currently no therapies for the treatment of these diseases, making this an area of active investigation.
In a study appearing in the December issue of the Journal of Clinical Investigation, Alexander Pfeifer and colleagues from the University of Bonn, Germany, show that in mice silencing of the gene encoding PrPc suppresses the accumulation of PrPsc. In vitro, silencing the gene encoding PrPc, using a technique known as RNA interference (RNAi), in already diseased neurons suppressed the accumulation of PrPsc. Similarly, in mice engineered to express the gene silencing therapeutic in a varying proportion of their neurons, the accumulation of PrPsc was markedly delayed, with the delay in accumulation of PrPsc being directly correlated with the proportion of neurons in the brain expressing the gene silencing therapeutic. This study therefore provides hope that RNAi might provide a new approach for the development of a therapeutic to treat individuals and animals with neurodegenerative disorders such as CJD and BSE. However, as Qingzhong Kong from Case Western Reserve University says in an accompanying commentary "Much more research is needed before RNAi can be harnessed to treat"¦" these neurodegenerative disorders.
Journal of Clinical Investigation
Mad Cow Disease News and Current Mad Cow Disease Events RSSEven before tomato warning, many Americans lacked confidence in the food safety system
A new national study conducted by the Harvard School of Public Health Project on the Public and Biological Security finds that, in spite of a number of food safety incidents in recent years, most Americans remain confident that the food produced in the United States is safe. However, many have concerns about the safety of imported food produced in some other countries.
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.
Deadly dose: Rensselaer heparin expert helps uncover source of lethal contamination
The mysterious death of patients around the world following a routine dosage of the common blood thinner, heparin, sent researchers on a frantic search to uncover what could make the standard drug so toxic.
Medical College of Wisconsin discovery alters longstanding concept of fixed protein structure
The thousands of proteins found in nature are simply strings of amino acids, assembled by genes, and scientists have long believed that they automatically fold themselves into uniquely fixed, 3-dimensional shapes to fire the engine of life.
Clemson scientists shed light on molecules in living cells
Clemson University chemists have developed a method to dramatically improve the longevity of fluorescent nanoparticles that may someday help researchers track the motion of a single molecule as it travels through a living cell.
Thermochemical process converts poultry litter into bio-oil
Foster Agblevor, associate professor of biological systems engineering, is leading the team of researchers in the College of Agriculture and Life Sciences at Virginia Tech developing transportable pyrolysis units that will convert poultry litter into bio-oil, providing an economical disposal system while reducing environmental effects and biosecurity issues.
New prion protein discovered by Canadian scientists may offer insight into mad cow disease
Scientists have discovered a new protein that may offer fresh insights into brain function in mad cow disease. "Our team has defined a second prion protein called 'Shadoo', that exists in addition to the well-known prion protein called 'PrP' " said Professor David Westaway, director of the Centre for Prions and Protein Folding Diseases at the University of Alberta.
Scientists identify prion's infectious secret
Researchers have known for decades that certain neurodegenerative diseases, such as mad cow disease or its human equivalent, Cruetzfeldt-Jakob disease, result from a kind of infectious protein called a prion.
Vaccine prevents prion disease in mice
An oral vaccine can prevent mice from developing a brain disease similar to mad cow disease.
The CReSA is working on a new strategy to combat spongiforms
Researchers at the Animal Health Research Centre (CReSA) are developing immunotherapeutical strategies against diseases produced by prion, such as Bovine Spongiform Encephalitis.
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