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Findings Could Advance Understanding Of Mad Cow Disease

16 June 2009

US - Scientists at the National Institutes of Health have gained a major insight into how the rogue protein responsible for mad cow disease and related neurological illnesses destroys healthy brain tissue.

"This advance sets the stage for future efforts to develop potential treatments for prion diseases or perhaps to prevent them from occurring," said Duane Alexander, M.D., Director of NIH’s Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), where the study was conducted.

The researchers discovered that the protein responsible for these disorders, known as prion protein (PrP), can sometimes wind up in the wrong part of a cell.

When this happens, PrP binds to Mahogunin, a protein believed to be essential to the survival of some brain cells. This binding deprives cells in parts of the brain of functional Mahogunin, causing them to die eventually. The scientists believe this sequence of events is an important contributor to the characteristic neurodegeneration of these diseases.

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