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
Related Documents :
10941149 - Areas of demyelination do not attract significant numbers of schwann cells transplanted...
7506619 - Changes in dna synthesis rate in the schwann cell lineage in vivo are correlated with t...
7276539 - The neurosecretory cells and retrocerebral endocrine glands of amsacta collaris hampson...
9742149 - Requirement for early-generated neurons recognized by monoclonal antibody lot1 in the f...
2627889 - Control of peripheral glial cell proliferation: enteric neurons exert an inhibitory inf...
10476679 - In vivo predegeneration of peripheral nerves: an effective technique to obtain activate...
16882139 - Influence of starvation, surface attachment and biofilm growth on the biocide susceptib...
16163699 - Signal transduction of erbb receptors in trastuzumab (herceptin) sensitive and resistan...
23901039 - Cell-autonomous and non-cell-autonomous mechanisms of hgf/met-driven resistance to targ...
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    
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
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

Previous Document:  cAMP-dependent axon guidance is distinctly regulated by Epac and protein kinase A.
Next Document:  Four projection streams from primate V1 to the cytochrome oxidase stripes of V2.