Prion Protein Keeps Nerves Happy

Prions (misshapen proteins) are notorious to wreak havoc in the brain when let loose, such as in mad cow disease. Believe it or not, Prions start life off as normal, healthy proteins called PrP. PrP is everywhere in the brain, which some scientists now say plays an important role in maintaining myelin. Eleven years ago, a Japanese group published a paper claiming that mice without PrP had damage to their peripheral nerves. Adriano Aguzzi, a neuropathologist was intrigued by this finding. First, his team examined five strains of mice missing the PrP gene. He found that all the mice showed this peripheral nerve damage by 10 weeks of age. Then, they studied mice that lacked PrP to see what caused the demyelination. When PrP was present only in axons, it prevented disease. But when it was lacking in axons but present in the Schwann cells the mice got sick. Aguzzi theorizes that PrP is a signaling molecule in axons that's needed to "keep Schwann cells happy." Aguzzi is now interested in learning if PrP might be part in human demyelinating diseases. Other human diseases "look very similar to what we see in these mice," notes Aguzzi.
J. Couzin-Frankel, “Prion Protein Keeps Nerves Happy,” Science, Jan 25, 2010. Retrieved on Feb 18, 2010: http://news.sciencemag.org/sciencenow/2010/01/25-04.html .
0 comments:
Post a Comment