( A role for prion proteins the much deb...)Prions Prevent Alzheimers Disease,Health

A role for prion proteins, the much debated agents of mad cow disease and vCJD, has been identified. It appears that the normal prions produced by the body help to prevent the plaques that build up in the brain to cause Alzheimers disease.

The possible function for the mysterious proteins was discovered by a team of scientists led by Medical Research Council funded scientist Professor Nigel Hooper of the University of Leeds.

Alzheimers and diseases like variant Creutzfeldt-Jakob Disease follow similar patterns of disease progression and in some forms of prion disease share genetic features. These parallels prompted Professor Hoopers team to look for a link between the different conditions. They found an apparent role for normal prion proteins in preventing Alzheimers disease.

Our experiments have shown that the normal prion proteins found in brain cells reduce the formation of beta-amyloid, a protein that binds with others to build plaques in the brain that are found in Alzheimers disease, explains Professor Hooper.

He continues: In vCJD, the normal version of prion protein, PrPc, found naturally in the brain is corrupted by infectious prions to cause disease. The normal function of PrPc has been unclear.

Using cells grown in the lab, the team looked at the effect of high and low levels of normal prion protein on the successful formation of beta amyloid, the source of Alzheimers plaques. They found that beta amyloid did not form in cells with higher than usual levels of PrPc. In comparison, when the level of PrPc was low or absent, beta amyloid formation was found to go back up again.

Mice genetically engineered to lack PrPc were also studied. Again, this revealed that in its absence, the harmful beta-amyloid proteins were able to form. It appears that PrPc, the normal prion protein, exerts its beneficial effect by stopping an enzyme called beta-secretase from cutting up amyloid protei
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