Association of a bovine prion gene haplotype with atypical BSE.
BACKGROUND
Atypical bovine spongiform encephalopathies (BSEs) are recently recognized prion diseases of cattle. Atypical BSEs are rare; approximately 30 cases have been identified worldwide. We tested prion gene (PRNP) haplotypes for an association with atypical BSE.


RESULTS
Haplotype tagging polymorphisms that characterize PRNP haplotypes from the promoter region through the three prime untranslated region of exon 3 (25.2 kb) were used to determine PRNP haplotypes of six available atypical BSE cases from Canada, France and the United States. One or two copies of a distinct PRNP haplotype were identified in five of the six cases (p = 1.3 x 10(-4), two-tailed Fisher's exact test; CI(95%) 0.263-0.901, difference between proportions). The haplotype spans a portion of PRNP that includes part of intron 2, the entire coding region of exon 3 and part of the three prime untranslated region of exon 3 (13 kb).


CONCLUSIONS
This result suggests that a genetic determinant in or near PRNP may influence susceptibility of cattle to atypical BSE.
Clawson
Michael L
ML
United States Department of Agriculture, Agricultural Research Service, US Meat Animal Research Center, Clay Center, Nebraska, United States of America. mike.clawson@ars.usda.gov
Richt
Juergen A
JA
Baron
Thierry
T
Biacabe
Anne-Gaëlle
AG
Czub
Stefanie
S
Heaton
Michael P
MP
Smith
Timothy P L
TP
Laegreid
William W
WW
D000818:Animals; D002417:Cattle; D016643:Encephalopathy, Bovine Spongiform / Q000235:genetics*; D005838:Genotype; D006239:Haplotypes; D011110:Polymorphism, Genetic; D011328:Prions / Q000235:genetics*
D011328:Prions