Document Detail

Resistance to scrapie in PrP ARR/ARQ heterozygous sheep is not caused by preferential allelic use.
MedLine Citation:
PMID:  15166274     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: In sheep, susceptibility to scrapie, which is similar to human prion diseases such as Kuru and variant Creutzfeldt-Jakob disease (vCJD), is determined by prion protein (PrP) gene (Prnp) polymorphisms. Sheep with genotype ARQ/ARQ, denoting polymorphisms at codons 136, 154, and 171, are susceptible, whereas those with genotypes ARR/ARQ and ARR/ARR are resistant, indicating dominance of ARR over the ARQ allele. AIMS: Based on familial CJD E200K, 129V, where preferential use of the 200E allele in EK heterozygous individuals confers resistance, heterozygous ARR/ARQ sheep were used to test the hypothesis that resistance is caused by preferential use of the ARR allele. METHODS: After assessment of equivalent PrP expression across genotypes, allele use was analysed by sequencing reverse transcription polymerase chain reaction derived DNA clones containing the Prnp gene coding sequence. RESULTS: The ARR to ARQ ratio was 1.1 in 133 clones, representing Prnp mRNA from three ARR/ARQ sheep, indicating equal use of both alleles. CONCLUSIONS: Dominance of the resistant associated allele in sheep scrapie involves mechanisms other than the absence of PrP derived from the disease associated ARQ allele.
P A Caplazi; K I O'Rourke; T V Baszler
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, Non-P.H.S.    
Journal Detail:
Title:  Journal of clinical pathology     Volume:  57     ISSN:  0021-9746     ISO Abbreviation:  J. Clin. Pathol.     Publication Date:  2004 Jun 
Date Detail:
Created Date:  2004-05-28     Completed Date:  2004-06-21     Revised Date:  2009-11-18    
Medline Journal Info:
Nlm Unique ID:  0376601     Medline TA:  J Clin Pathol     Country:  England    
Other Details:
Languages:  eng     Pagination:  647-50     Citation Subset:  AIM; IM    
Department of Veterinary Microbiology and Pathology, College of Veterinary Medicine, Washington State University, Pullman, WA 99164-7040, USA.
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MeSH Terms
Fluorescent Antibody Technique
Genetic Predisposition to Disease
Prions / genetics*,  metabolism
Scrapie / genetics*,  metabolism
Reg. No./Substance:

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