Document Detail

CRBL cells: establishment, characterization and susceptibility to prion infection.
MedLine Citation:
PMID:  18395703     Owner:  NLM     Status:  MEDLINE    
The cerebellum is involved in complex physiological functions including motor control, sensory perception, cognition, language, and emotion. Humans and animals with prion diseases are characterized clinically by ataxia, postural abnormalities and cognitive decline. Pathology in the cerebellum affected by prions includes spongiform degeneration, neuronal loss, and gliosis. To develop an in vitro model system for studying prion biology in cerebellar cells, we established and characterized an immortal cell line (CRBL) isolated from the cerebellum of mice lacking expression of a protein involved in cell cycle arrest. The characteristics of the cells include morphological heterogeneity, rapid proliferation, serum responsiveness during growth, and a change in the number of chromosomes. CRBL cells expressed both neuronal and glial cell markers as well as a considerable level of cellular prion protein, PrP(C). Upon in vitro infection, CRBL cells exhibited selective susceptibility to prions isolated from different sources. These cells chronically propagated prions from SMB cells. Strain-specific prion infection in CRBL cells was not due to instability of the cell line, allelic variance, or mutations in the PrP gene. Molecular properties of prions derived from SMB cells were maintained in the infected CRBL cells. Our results suggest that the specific interaction between a prion strain and hosts determined the selective susceptibility of CRBL cells, which reflects the conditions in vivo. In addition to the future studies revealing cellular and molecular mechanism involved in prion pathogenesis, CRBL cells will contribute to the studies dealing with prion strain properties and host susceptibilities.
Charles E Mays; Hae-Eun Kang; Younghwan Kim; Sung Han Shim; Ji-Eun Bang; Hee-Jong Woo; Youl-Hee Cho; Jae-Beom Kim; Chongsuk Ryou
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Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't     Date:  2008-03-18
Journal Detail:
Title:  Brain research     Volume:  1208     ISSN:  0006-8993     ISO Abbreviation:  Brain Res.     Publication Date:  2008 May 
Date Detail:
Created Date:  2008-04-25     Completed Date:  2008-10-02     Revised Date:  2014-09-08    
Medline Journal Info:
Nlm Unique ID:  0045503     Medline TA:  Brain Res     Country:  Netherlands    
Other Details:
Languages:  eng     Pagination:  170-80     Citation Subset:  IM    
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MeSH Terms
Cell Count
Cell Line, Transformed / physiology*
Cells, Cultured
Cerebellum / cytology
Cytogenetics / methods
Disease Susceptibility*
Flow Cytometry
Glial Fibrillary Acidic Protein / metabolism
Mice, Knockout
Neurons / physiology*
Prions / metabolism*
Transfection / methods
Tubulin / metabolism
Tumor Suppressor Protein p53 / deficiency
Grant Support
P20 RR 020171/RR/NCRR NIH HHS; P20 RR020171/RR/NCRR NIH HHS; P20 RR020171-03/RR/NCRR NIH HHS
Reg. No./Substance:
0/Glial Fibrillary Acidic Protein; 0/Prions; 0/Tubulin; 0/Tumor Suppressor Protein p53; 0/beta3 tubulin, mouse

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

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