Monday 10 March 2014

New way to treat prion diseases

A research development reveals natural mechanism in brain cells that protects against “molecular piracy” of deadly neurological diseases.

Prion diseases lead to incurable neurodegenerative disorders such as Creutzfeldt-Jakob disease in humans, mad cow disease (bovine spongiform encephalopathy) and chronic wasting disease in deer and elk.

Research, published in The Journal of Clinical Investigation, stemmed from two largely unexplained factors for prion infections: their long incubation periods of up to five decades, and a noted time discrepancy between the accumulation of the misfolded rogue prion protein in the brain and when the disease actually shows symptoms.

In probing these mysteries, Westaway, U of A post-doctoral fellow Charles E. Mays, associate professor Jiri Safar of Case Western Reserve University and other international collaborating researchers began two years ago to study a molecule called the “shadow” of the prion protein. Significant changes in this shadow protein have directed researchers to examine the normal prion protein itself.

For further details, see the paper “Prion disease tempo determined byhost-dependent substrate reduction”.

Posted by Tim Sandle

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