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After 20 years of research, scientists believe they have finally uncovered the normal function of prion proteins, which can cause deadly illnesses such as Creutzfeldt–Jakob disease (CJD) if they become incorrectly folded. An international team of neuroscientists reports that, in mammals, the mysterious proteins help to maintain the myelin sheath that protects the body's nerves. "This opens a new door to studying some of the many common neuropathy disorders — which lead to weakness or loss of sensitivity of limbs — where we don't know the cause," says prion expert Simon Mead at University College London's Institute of Neurology.
The authors suspect that their finding also applies to brain neurons. If so, this would have implications for treating deadly CJD and other transmissible spongiform encephalopathies. It could also offer a new way of looking at multiple sclerosis, an incurable disease caused by demyelination of nerves in the brain and spinal cord. The work is published online in Nature Neuroscience.
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