Document Detail

Identification and characterization of a novel mouse prion gene allele.
MedLine Citation:
PMID:  15170227     Owner:  NLM     Status:  MEDLINE    
The major determinant of prion disease incubation time in mice is thought to be the amino acid sequence of the prion protein. Two alleles of the mouse prion gene ( Prnp) have been described, where Prnp(a) (Leu-108, Thr-189) and Prnp(b) (Phe-108, Val-189) are associated with short and long incubation times, to defined prion strains, respectively. As part of a survey of inbred mouse lines, the prion gene open reading frame was sequenced and revealed a new allele, Prnp(c) (Phe-108, Thr-189), in the strain MAI/Pas. To study the influence of Prnp(c) independently of the MAI/Pas genetic background, we generated a congenic line in which Prnp(c) was bred onto the C57BL/6JOlaHsd background. Following intracerebral inoculation with Chandler/RML scrapie prions, the congenic mice showed an increased mean incubation time relative to C57BL/6JOlaHsd, of over 100 days. However, no differences were observed in the intensity and pattern of PrP immunoreactivity deposition or spongiosis. We conclude that the new allele, Prnp(c), modulates incubation time but not neuropathology and that the previous classification of mice into two distinct groups based on incubation time and Prnp genotype should now be revised.
Sarah E Lloyd; Simon R Thompson; Jonathan A Beck; Jacqueline M Linehan; Jonathan D F Wadsworth; Sebastian Brandner; John Collinge; Elizabeth M C Fisher
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Mammalian genome : official journal of the International Mammalian Genome Society     Volume:  15     ISSN:  0938-8990     ISO Abbreviation:  Mamm. Genome     Publication Date:  2004 May 
Date Detail:
Created Date:  2004-05-31     Completed Date:  2004-11-06     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  9100916     Medline TA:  Mamm Genome     Country:  United States    
Other Details:
Languages:  eng     Pagination:  383-9     Citation Subset:  IM    
MRC Prion Unit and Department of Neurodegenerative Disease, Institute of Neurology, University College, London, WC1N 3BG, UK.
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MeSH Terms
Crosses, Genetic
Mice, Inbred C57BL
Mice, Transgenic
Neurons / metabolism*,  pathology
Prions / chemistry,  physiology*
Scrapie / etiology*
Species Specificity
Structure-Activity Relationship
Time Factors
Reg. No./Substance:

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