Biological and biochemical characterization of L-type-like bovine spongiform encephalopathy (BSE) detected in Japanese black beef cattle

Prion. 2008 Jul-Sep;2(3):123-8. doi: 10.4161/pri.2.3.7437. Epub 2008 Jul 14.

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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Blotting, Western
  • Cattle
  • Encephalopathy, Bovine Spongiform / diagnosis*
  • Encephalopathy, Bovine Spongiform / metabolism*
  • Encephalopathy, Bovine Spongiform / transmission
  • Endopeptidase K / metabolism
  • Glycoproteins / metabolism
  • Immunohistochemistry
  • Mice
  • Mice, Transgenic
  • Molecular Weight
  • Nervous System / pathology
  • Positron-Emission Tomography
  • PrPSc Proteins / metabolism
  • Protein Conformation
  • Protein Stability

Substances

  • Glycoproteins
  • PrPSc Proteins
  • Endopeptidase K