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Neurodevelopmental expression and localization of the cellular prion protein in the central nervous system of the mouse

Transmissible spongiform encephalopathies (TSEs) are neurodegenerative disorders caused by PrPSc, or prion, an abnormally folded form of the cellular prion protein (PrPC). The abundant expression of PrPC in the central nervous system (CNS) is a requirement for prion replication, yet despite years of intensive research the physiological function of PrPC still remains unclear. Several routes of investigation point out a potential role for PrPC in axon growth and neuronal development. Thus, we undertook a detailed analysis of the spatial and temporal expression of PrPC during mouse CNS development. Our findings show regional differences of the expression of PrP, with some specific white matter structures showing the earliest and highest expression of PrPC. Indeed, all these regions are part of the thalamolimbic neurocircuitry, suggesting a potential role of PrPC in the development and functioning of this specific brain system. J. Comp. Neurol. 518:1879–1891, 2010. © 2010 Wiley‐Liss, Inc.