Mutant Proteins Result In Infectious Prion Disease In MiceA worldwide group of scientists has created an infectious prion disease in a mouse model, in a step that may help unravel the mystery of this progressive disease that affects the nervous system in humans and animals. The research team, including Christina J. Sigurdson, D.V.M., Ph.D.
Prion Infectivity Found In White And Brown Fat Tissues Of MiceResearchers from the National Institutes of Health and the Scripps Research Institute have found novel prion infectivity in white and brown fat tissues of mice. The study appears December 5 in the open-access journal PLoS Pathogens. Prion diseases, also known as transmissible spongiform encephalopathies, are infectious progressive fatal neurodegenerative diseases which affect humans as well as wild and domestic animals.
FDA November 2008 Update On Feed Enforcement Activities To Limit The Spread Of BSETo help prevent the establishment and amplification of Bovine Spongiform Encephalophathy (BSE) through feed in the United States, the Food and Drug Administration (FDA) implemented a final rule that prohibits the use of most mammalian protein in feeds for ruminant animals. This rule, Title 21 Part 589.2000 of the Code of Federal Regulations, here called the Ruminant Feed Ban, became effective on August 4, 1997.
Variant CJD And Blood TransfusionUntil recently the risk of developing CJD as a consequence of a blood transfusion was a theoretical concern. However, in December 2003 a patient died from vCJD after receiving a blood transfusion from a donor who subsequently also had vCJD. Since then, three further patients have been identified. One patient died of an unrelated condition, but a post mortem examination established that the abnormal form of the prion protein was present in their body.
Hot On The Heels Of The Mad Cow Pathogen - First Synthetic Anchored PrionIn the mid-90s, mad cow disease was on everyone's lips and the front page of every newspaper. What made the epizootic outbreak so alarming was the as-sumption that a mutation of the fatal Creutzfeld Jakob Disease (vCJD) could be triggered in humans through the consumption of BSE-infected beef. In both species, the diseases result in brain degeneration. For quite some time, re-search has proceeded on the assumption that wrongly folded prions are re-sponsible.
FSA Supports Move To Increase The Age At Which UK Cattle Are BSE Tested From 30 To 48 MonthsThe Food Standards Agency (FSA) Board today agreed to support a move to increase the age at which UK cattle are BSE tested from 30 months to 48 months, subject to a review of current and continued BSE surveillance. FSA Chair, Dame Deirdre Hutton, said: 'The Board supports the move to testing at 48 months but would not wish this to be implemented until a further report on surveillance has been produced and this has been passed to SEAC for review.
Seeking The Cause Of Mad Cow DiseaseThe cause of diseases such as BSE in cattle and Creutzfeld-Jakob disease in humans is a prion protein. This protein attaches to cell membranes by way of an anchor made of sugar and lipid components (a glycosylphosphatidylinositol, GPI) anchor. The anchoring of the prions seems to have a strong influence on the transformation of the normal form of the protein into its pathogenic form, which causes scrapie and mad cow disease. A team headed by Christian F. W.
Is There More To Prion Protein Than Mad Cow Disease?Prion protein, a form of protein that triggers BSE, is associated with other brain diseases in cattle, raising the possibility of a significant increase in the range of prion disease. Publishing their findings in the open access journal BMC Veterinary Research, scientists have detected changes in the production and accumulation of the prion protein in the brains of cattle with a rare neurodegenerative disorder.
When It Comes To Prions, Protective Pathway In Stressed Cells Not So HelpfulScientists at the National Institutes of Health (NIH) have discovered that an important cellular quality control mechanism may actually be toxic to some brain cells during prion infection. The research, published by Cell Press in the September 16th issue of the journal Developmental Cell, proposes a new general mechanism of cellular dysfunction that can contribute to the devastating and widespread neuronal death characteristic of slowly progressing neurodegenerative diseases.
Mad Cow Disease Also Caused By Genetic Mutation Research ShowsNew findings about the causes of mad cow disease show that sometimes it may be genetic. "We now know it's also in the genes of cattle," said Juergen A. Richt, Regents Distinguished Professor of Diagnostic Medicine and Pathobiology at Kansas State University's College of Veterinary Medicine. Until several years ago, Richt said, it was thought that the cattle prion disease bovine spongiform encephalopathy -- also called BSE or mad cow disease -- was a foodborne disease.
Jumping The 'Species Barrier' - Infectious, Test Tube-Produced PrionsResearchers have shown that they can create entirely new strains of infectious proteins known as prions in the laboratory by simply mixing infectious prions from one species with the normal prion proteins of another species. The findings are reported in the September 5th issue of the journal Cell, a Cell Press publication.
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