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Published on: April 13, 2017
Neurodevelopmental expression and localization of the cellular prion protein in the central nervous system of the
Stefano Benvegnù1, Ilaria Poggiolini, Giuseppe Legname
1Scuola Internazionale Superiore di Studi Avanzati-International School for Advanced Studies (SISSA-ISAS), Neurobiology Sector, I-34151 Trieste, Italy.
Abstract:
Transmissible spongiform encephalopathies (TSEs) are neurodegenerative disorders caused by PrP(Sc), or prion, an abnormally folded form of the cellular prion protein (PrP(C)). The abundant expression of PrP(C) in the central nervous system (CNS) is a requirement for prion replication, yet despite years of intensive research the physiological function of PrP(C) still remains unclear. Several routes of investigation point out a potential role for PrP(C) in axon growth and neuronal development. Thus, we undertook a detailed analysis of the spatial and temporal expression of PrP(C) 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 PrP(C). Indeed, all these regions are part of the thalamolimbic neurocircuitry, suggesting a potential role of PrP(C) in the development and functioning of this specific brain system.
