Biological and biochemical characterization of L-type-like bovine spongiform encephalopathy (BSE) detected in Japanese black beef cattle.
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.
Masujin
Kentaro
K
Prion Disease Research Center, National Institute of Animal Health, Tsukuba, Ibaraki, Japan.
Shu
Yujing
Y
Yamakawa
Yoshio
Y
Hagiwara
Ken'ichi
K
Sata
Tetsutaro
T
Matsuura
Yuichi
Y
Iwamaru
Yoshifumi
Y
Imamura
Morikazu
M
Okada
Hiroyuki
H
Mohri
Shirou
S
Yokoyama
Takashi
T
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
D006023:Glycoproteins; D018620:PrPSc Proteins; D019286:Endopeptidase K