Neuroprotective functions of prion protein
Version of Record online: 24 NOV 2003
Copyright © 2003 Wiley-Liss, Inc.
Journal of Neuroscience Research
Volume 75, Issue 2, pages 153–161, 15 January 2004
How to Cite
Roucou, X., Gains, M. and LeBlanc, A. C. (2004), Neuroprotective functions of prion protein. J. Neurosci. Res., 75: 153–161. doi: 10.1002/jnr.10864
- Issue online: 30 DEC 2003
- Version of Record online: 24 NOV 2003
- Manuscript Accepted: 9 OCT 2003
- Manuscript Revised: 7 OCT 2003
- Manuscript Received: 4 AUG 2003
The normal function of prion protein (PrP) is usually disregarded at the expense of the more fascinating role of PrP in transmissible prion diseases. However, the normal PrP may play an important role in cellular function in the central nervous system, since PrP is highly expressed in neurons and motifs in the sequence of PrP are conserved in evolution. The finding that prion null mice do not have a significant overt phenotype suggests that the normal function of PrP is of minor importance. However, the absence of PrP in cells or in vivo contributes to an increased susceptibility to oxidative stress or apoptosis-inducing insults. An alternative explanation is that the PrP normal function is so important that it is redundant. Probing into the characteristics of PrP has revealed a number of features that could mediate important cellular functions. The neuroprotective actions so far identified with PrP are initiated through cell surface signaling, antioxidant activity, or anti-Bax function. Here, we review the characteristics of the PrP and the evidence that PrP protects against neurodegeneration and neuronal cell death. © 2003 Wiley-Liss, Inc.