New findings made in prion disease studies

June 11, 2009

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 prion proteins, can sometimes settle in the wrong part of a cell. When that happens, the prion protein binds to Mahogunin, a protein believed to be essential to the survival of some brain cells. That binding, researchers said, deprives some brain cells of functional Mahogunin, causing them to eventually die.

The scientists believe that sequence of events is an important contributor to the characteristic neurodegeneration caused by prion diseases such as mad cow.

The study appears in the current issue of the journal Cell.

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