title: Association of a bovine prion gene haplotype with atypical BSE.
abstract: 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.
authors: 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
authors: Michael L
authors: ML
authors: Clawson
authors: Juergen A
authors: JA
authors: Richt
authors: Thierry
authors: T
authors: Baron
authors: Anne-Gaëlle
authors: AG
authors: Biacabe
authors: Stefanie
authors: S
authors: Czub
authors: Michael P
authors: MP
authors: Heaton
authors: Timothy P L
authors: TP
authors: Smith
authors: William W
authors: WW
authors: Laegreid
mesh_terms: D000818:Animals; D002417:Cattle; D016643:Encephalopathy, Bovine Spongiform / Q000235:genetics*; D005838:Genotype; D006239:Haplotypes; D011110:Polymorphism, Genetic; D011328:Prions / Q000235:genetics*
chemical_list: D011328:Prions