Document Detail

Dramatic reduction of PrP C level and glycosylation in peripheral nerves following PrP knock-out from Schwann cells does not prevent transmissible spongiform encephalopathy neuroinvasion.
MedLine Citation:
PMID:  20007469     Owner:  NLM     Status:  MEDLINE    
Expression of the prion protein (PrP(C)) is a requirement for host susceptibility to the transmissible spongiform encephalopathies (TSEs) and thought to be necessary for the replication and transport of the infectious agent. The mechanism of TSE neuroinvasion is not fully understood, although the routing of infection has been mapped through the peripheral nervous system (PNS) and Schwann cells have been implicated as a potential conduit for transport of the TSE infectious agent. To address whether Schwann cells are a requirement for spread of the TSE agent from the site of infection to the CNS, PrP(C) expression was selectively removed from Schwann cells in vivo. This dramatically reduced total PrP(C) within peripheral nerves by 90%, resulting in the selective loss of glycosylated PrP(C) species. Despite this, 139A and ME7 mouse-passaged scrapie agent strains were efficiently replicated and transported to the CNS following oral and intraperitoneal exposure. Thus, the myelinating glial cells within the PNS do not appear to play a significant role in TSE neuroinvasion.
Barry M Bradford; Nadia L Tuzi; M Laura Feltri; Caroline McCorquodale; Enrico Cancellotti; Jean C Manson
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  The Journal of neuroscience : the official journal of the Society for Neuroscience     Volume:  29     ISSN:  1529-2401     ISO Abbreviation:  J. Neurosci.     Publication Date:  2009 Dec 
Date Detail:
Created Date:  2009-12-16     Completed Date:  2010-01-19     Revised Date:  2014-09-20    
Medline Journal Info:
Nlm Unique ID:  8102140     Medline TA:  J Neurosci     Country:  United States    
Other Details:
Languages:  eng     Pagination:  15445-54     Citation Subset:  IM    
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MeSH Terms
Brain / pathology,  physiopathology
Infectious Disease Incubation Period
Kaplan-Meier Estimate
Mice, Knockout
Mice, Transgenic
Peripheral Nerves / pathology,  physiopathology*
PrPC Proteins / genetics,  metabolism*
PrPSc Proteins / metabolism
Prion Diseases / pathology,  physiopathology*,  transmission*
Schwann Cells / pathology,  physiology*
Sciatic Nerve / pathology,  physiopathology
Scrapie / pathology,  physiopathology,  transmission
Time Factors
Vacuoles / pathology,  physiology
Grant Support
G9721848/D42740//Medical Research Council; R01 NS045630/NS/NINDS NIH HHS; R01 NS045630-06/NS/NINDS NIH HHS; R01 NS045630-07/NS/NINDS NIH HHS; R01 NS045630-08/NS/NINDS NIH HHS; R01 NS045630-09/NS/NINDS NIH HHS; RI/ISPG3//Biotechnology and Biological Sciences Research Council
Reg. No./Substance:
0/PrPC Proteins; 0/PrPSc Proteins

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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