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Prions play vital role in nerve health

26th January 2010

New Swiss experiments suggest that the prion proteins that lead to Creutzfeldt-Jakob disease (vCJD), a human variant of Mad Cow Disease (BSE) may actually play an important role in the nervous system.

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Their research suggests that the 'misfolded' proteins help maintain the myelin sheath that surrounds nerves and brain cells.

Prion expert Simon Mead at University College London's Institute of Neurology said that the finding opens the door to the study of some mysterious disorders.

The authors of the study think that their research may lead to new avenues for treating CJD and even multiple sclerosis (MS), a currently incurable disease affecting the myelin sheath around nerves in the brain and spinal cord.

Study leader Adriano Aguizzi of the University Hospital of Zurich in Switzerland said that, since 1991, scientists had been studying the effects of prions in different animals.

He said that it had been difficult to draw any conclusions from the initial studies, and that researchers came to associate a lack of prions with prion immunity.

The recent finding overturns that belief, finding evidence that prions may play a vital role in maintaining nerve cells, and that an absence of prions causes diseases of the peripheral nervous system.

For the purposes of the study, the scientists examined a breed of mice known to be resistant to prion diseases, the equivalent of vCJD in their own species.

The mice were abnormal in many ways.

The nerve cells connecting their limbs and organs to the spinal cord and brain tended to degenerate as they aged, as did the myelin sheath corresponding to those nerves.

Further investigations into the removal of the prion protein showed that, when it was removed from the cells responsible for making the myelin sheath, there was no effect upon the degeneration of the nerve cells.

However, removing the prion from the nerve cells themselves was what led to the degeneration the researchers had observed initially.

The researchers said that, now that they understand that prion proteins play some role in maintaining healthy nerve cells in the brain and spinal cord, they will have new avenues to explore for disease treatment.

However, they are still uncertain as to which human disorders would correspond to their experiments in mice.

Nigel Hooper of the University of Leeds, who has done research on prions in connection with Alzheimer's disease, said that he believed there was some evidence that prions could have a number of different roles.

He said that the prion's role would depend on its whereabouts in the body.


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