title: Bovine spongiform encephalopathy in Sweden: an H-type variant.
abstract: Bovine spongiform encephalopathy (BSE) had never been detected in Sweden until 2006, when the active surveillance identified a case in a 12-year-old cow. The case was an unusual form, because several molecular features of the protease-resistant prion protein (PrP(res)) were different from classical BSE. The differences included higher susceptibility for proteinase K, higher molecular weight of the PrP(res) bands, affinity to the N-terminus-specific antibodies 12B2 and P4, and peculiar banding pattern with antibody SAF84 showing an additional band at the 14 kDa position. The molecular characteristics were in accordance to previous descriptions of H-type BSE. This report shows that a range of Western blot techniques and antibodies can be applied to confirm H-type BSE and further describes that the ratio of the amounts of PrPres#1 and PrPres#2, after deglycosylation, depends on the antibody used during processing. Immunohistochemistry on sections of medulla at the level of the obex applying antibodies with epitopes covering a broad range of the PrP sequence showed accumulation of disease-specific PrP (PrP(d)) in the gray matter. Fine punctate deposition in the neuropil was the most predominant type and was more severe in BSE target nuclei. The types of PrP(d) deposition are described in comparison with classical BSE. PrP-gene sequencing showed 6 copy octarepeat alleles and no abnormalities. It is postulated that the disease had a spontaneous origin, rather than having had been acquired in the BSE epidemic.
authors.affiliation: National Veterinary Institute, S-75189 Uppsala, Sweden. dolores@sva.se
authors.forename: Dolores
authors.initials: D
authors.lastname: Gavier-Widén
authors.forename: Maria
authors.initials: M
authors.lastname: Nöremark
authors.forename: Jan P M
authors.initials: JP
authors.lastname: Langeveld
authors.forename: Mick
authors.initials: M
authors.lastname: Stack
authors.forename: Anne-Gaëlle
authors.initials: AG
authors.lastname: Biacabe
authors.forename: Johann
authors.initials: J
authors.lastname: Vulin
authors.forename: Melanie
authors.initials: M
authors.lastname: Chaplin
authors.forename: Jürgen A
authors.initials: JA
authors.lastname: Richt
authors.forename: Jorg
authors.initials: J
authors.lastname: Jacobs
authors.forename: Cristina
authors.initials: C
authors.lastname: Acín
authors.forename: Eva
authors.initials: E
authors.lastname: Monleón
authors.forename: Lena
authors.initials: L
authors.lastname: Renström
authors.forename: Berndt
authors.initials: B
authors.lastname: Klingeborn
authors.forename: Thierry G M
authors.initials: TG
authors.lastname: Baron
mesh_terms: D000818:Animals; D015153:Blotting, Western / Q000662:veterinary; D002417:Cattle; D016643:Encephalopathy, Bovine Spongiform / Q000453:epidemiology / Q000378:metabolism* / Q000473:pathology; D005260:Female; D014644:Genetic Variation; D005838:Genotype; D007150:Immunohistochemistry / Q000662:veterinary; D011110:Polymorphism, Genetic; D011247:Pregnancy; D011328:Prions / Q000235:genetics / Q000378:metabolism*; D013548:Sweden / Q000453:epidemiology
chemical_list: D011328:Prions