Category: CJD / vCJD / Mad Cow Disease

Burying prion-infected carcasses of cattle, deer and other animals in lime may actually enhance the spread of those infectious proteins through soil, a new study suggests. Placing quicklime on carcasses once was thought to be the best way to foster quick decay of bodies and to prevent the spread of disease. The study is scheduled for the April 15 issue of ACS’ Environmental Science & Technology, a semi-monthly journal.In the study, Joel A.

Studies in mice have indicated that the effects of prion disease could be reversed if caught early enough. The researchers said that their findings support developing early treatments that aim to reduce levels of prion protein in the brains of people with prion disease. Also, they said that their findings suggest testing the efficacy of treatments in a new way: by analyzing their cognitive effects in prion-infected mice.

The Food Standards Agency, UK, has been notified that an Over Thirty Month (OTM) heifer has entered the food chain without being tested for BSE. Testing of bovine animals is mandatory in those intended for human consumption that are over thirty months at slaughter.The heifer had its specified risk material removed, that is. those parts of the animal that would contain more than 99% of any infectivity that would be present if the animal had BSE.

The U.S. Food and Drug Administration is proposing to limit the materials used in some medical products in order to keep them free of the agent thought to cause mad cow disease, also known as bovine spongiform encephalopathy or BSE.This is the latest in a series of BSE safeguards that would bar material that has been found to harbor the highest concentrations of this fatal agent in infected cattle.

Scientists from the US and Japan have bred a dozen calves that don’t have prions - the infectious proteins that cause mad cow disease.The research project is reported in the online journal Nature Biotechnology.Preliminary tests suggest that the brains of the genetically engineered calves are immune to Bovine Spongiform Encephalopathy (BSE, or mad cow disease).

A filter that takes prions (carriers of vCJD) out of infected blood has been successfully tested on hamsters and UK scientists are optimistic of getting similar results with human donated blood.The results of this research are reported in The Lancet.

Post mortem examination of a third person infected with variant Creutzfeldt-Jakob Disease (vCJD) through blood transfusion has confirmed the presence of the fatal disease.Professor John Collinge of the Medical Research Council (MRC) Prion Unit has been tracking 66 people known to have accidentally received infected blood by transfusion. Of these, three are now confirmed to have died from vCJD and 39 from unrelated causes.

BSE (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.

A new method of treatment can appreciably slow down the progress of the fatal brain disease scrapie in mice. This has been established by researchers from the Universities of Munich and Bonn together with their colleagues at the Max Planck Institute in Martinsried. To do this they used an effect discovered by the US researchers Craig Mello and Andrew Fire, for which they were awarded this year’s Nobel Prize for Medicine.

The urgent search for a medication to treat prion diseases has led scientists in Germany to synthesize a new group of compounds, including one that is 15 times more potent than an approved drug now being tested in clinical trials. Their report is scheduled for the Nov. 2 issue of the biweekly ACS Journal of Medicinal Chemistry.Prions are infectious proteins that cause brain disorders like Mad Cow Disease and Creutzfeldt-Jakob Disease (CJD) in humans.