Abstract
Besides the classical form of bovine spongiform encephalopathy (BSE) that has been known for almost three decades, two atypical forms designated H-type and L-type BSE have recently been described. While the main diagnostic feature of these forms is the altered biochemical profile of the accumulated PrPSc, it was also observed in the initial analysis that L-type BSE displays a distribution pattern of the pathological prion protein (PrPSc), which clearly differs from that observed in classical BSE (C-type). Most importantly, the obex region in the brainstem is not the region with the highest PrPSc concentrations, but PrPSc is spread more evenly throughout the entire brain. A similar distribution pattern has been revealed for H-type BSE by rapid test analysis. Based on these findings, we performed a more detailed Western blot study of the anatomical PrPSc distribution pattern and the biochemical characteristics (molecular mass, glycoprofile as well as PK sensitivity) in ten different anatomical locations of the brain from cattle experimentally challenged with H- or L-type BSE, as compared to cattle challenged with C-type BSE. Results of this study revealed distinct differences in the PrPSc deposition patterns between all three BSE forms, while the biochemical characteristics remained stable for each BSE type among all analysed brain areas.
Highlights
Bovine spongiform encephalopathy (BSE) belongs to the so called ‘‘prion diseases’’, named transmissible spongiform encephalopathies (TSEs)
This study was aimed at the detailed analysis of the anatomical distribution and the biochemical characteristics of both atypical BSE forms designated L-type and H-type, as compared to C-type BSE
With a proteinase K (PK) concentration 20 times higher than what is normally used (i.e. 1000 mg/ml), we found a reduction in the signal intensity of approximately 50% for CType BSE, whereas the signals for both atypical BSE types were reduced by 93–97%, showing a residual signal intensity of only 3– 7% compared to that of the signals obtained after incubation with 50 mg/ml PK
Summary
Bovine spongiform encephalopathy (BSE) belongs to the so called ‘‘prion diseases’’, named transmissible spongiform encephalopathies (TSEs). These progressive neurodegenerative diseases are always fatal and can affect humans and animals. In 2004, two different, so called atypical BSE strains were described in cattle in France [3] and Italy [4]. They were designated H- and L-type BSE, due to the molecular weight of the disease-associated prion-protein (PrPSc) after protease degradation and Western blot analysis. Atypical BSE has been detected in more than 60 animals that were over eight years old in many countries across Europe, and in Canada, the United States and Japan [5]
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