title: Biological and biochemical characterization of L-type-like bovine spongiform encephalopathy (BSE) detected in Japanese black beef cattle.
abstract: A case of L-type-like atypical bovine spongiform encephalopathy was detected in 14-year-old Japanese black beef cattle (BSE/JP24). To clarify the biological and biochemical properties of the prion in BSE/JP24, we performed a transmission study with wild-type mice and bovinized transgenic mice (TgBoPrP). The BSE/JP24 prion was transmitted to TgBoPrP mice with the incubation period of 199.7 +/- 3.4 days, which was shorter than that of classical BSE (C-BSE) (223.5 +/- 13.5 days). Further, C-BSE was transmitted to wild-type mice with the incubation period of about 409 days, whereas BSE/JP24 prion inoculated mice showed no clinical signs up to 649 days. Severe vacuolation and a widespread and uniform distribution of PrP(Sc) were pathologically observed in the brain of BSE/JP24 prion affected TgBoPrP mice. The molecular weight and glycoform ratio of PrP(Sc) in BSE/JP24 were different from those in C-BSE, and PrP(Sc) in BSE/JP24 exhibited weaker proteinase K resistance than that in C-BSE. These findings revealed that the BSE/JP24 prion has distinct biological and biochemical properties reported for that of C-BSE. Interestingly, a shorter incubation period was observed at the subsequent passage of the BSE/JP24 prion to TgBoPrP mice (152.2 +/- 3.1 days). This result implies that BSE/JP24 prion has newly emerged and showed the possibility that L-type BSE prion might be classified into multiple strains.
authors.affiliation: Prion Disease Research Center, National Institute of Animal Health, Tsukuba, Ibaraki, Japan.
authors.forename: Kentaro
authors.initials: K
authors.lastname: Masujin
authors.forename: Yujing
authors.initials: Y
authors.lastname: Shu
authors.forename: Yoshio
authors.initials: Y
authors.lastname: Yamakawa
authors.forename: Ken'ichi
authors.initials: K
authors.lastname: Hagiwara
authors.forename: Tetsutaro
authors.initials: T
authors.lastname: Sata
authors.forename: Yuichi
authors.initials: Y
authors.lastname: Matsuura
authors.forename: Yoshifumi
authors.initials: Y
authors.lastname: Iwamaru
authors.forename: Morikazu
authors.initials: M
authors.lastname: Imamura
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Okada
authors.forename: Shirou
authors.initials: S
authors.lastname: Mohri
authors.forename: Takashi
authors.initials: T
authors.lastname: Yokoyama
mesh_terms: D000818:Animals; D015153:Blotting, Western; D002417:Cattle; D016643:Encephalopathy, Bovine Spongiform / Q000175:diagnosis* / Q000378:metabolism* / Q000635:transmission; D019286:Endopeptidase K / Q000378:metabolism; D006023:Glycoproteins / Q000378:metabolism; D007150:Immunohistochemistry; D051379:Mice; D008822:Mice, Transgenic; D008970:Molecular Weight; D009420:Nervous System / Q000473:pathology; D049268:Positron-Emission Tomography; D018620:PrPSc Proteins / Q000378:metabolism; D011487:Protein Conformation; D055550:Protein Stability
chemical_list: D006023:Glycoproteins; D018620:PrPSc Proteins; D019286:Endopeptidase K