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Neurodevelopmental expression and localization of the cellular prion protein in the central nervous system of the mouse
Author(s) -
Benvegnù Stefano,
Poggiolini Ilaria,
Legname Giuseppe
Publication year - 2010
Publication title -
journal of comparative neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.855
H-Index - 209
eISSN - 1096-9861
pISSN - 0021-9967
DOI - 10.1002/cne.22357
Subject(s) - biology , central nervous system , neuroscience , prion protein , white matter , axon , nervous system , axon guidance , microbiology and biotechnology , pathology , disease , medicine , radiology , magnetic resonance imaging
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. J. Comp. Neurol. 518:1879–1891, 2010. © 2010 Wiley‐Liss, Inc.