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The World in Medicine |

Prions Illuminated

Joan Stephenson, PhD
JAMA. 2010;303(7):603-603. doi:10.1001/jama.2010.149
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Researchers have long pondered the function of normal prion protein (PrPc), the nonpathogenic counterpart of the misfolded protein associated with such deadly disorders as bovine spongiform encephalopathy and Creutzfeldt-Jakob disease. Now, studies in mice by European scientists suggest that PrPc plays an important role in preserving the myelin sheath that protects the axons of peripheral nerves (Bremer J et al. Nature Neurosci. doi:10.1038/nn.2483 [published online January 24, 2010]).

The researchers found that several strains of mice that lack the gene encoding PrPc developed myelin damage by about 10 weeks of life. They also discovered that PrPc produced in neurons, but not PrPc produced in the Schwann cells, could prevent demyelination.

Clarifying the molecular basis for how neuron-expressed PrPc signals to Schwann cells to produce myelin “might lead to a better understanding of peripheral neuropathies—particularly those of late onset—and might help to uncover new therapeutic targets for these common, debilitating disorders,” the researchers noted.

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