H-type bovine spongiform encephalopathy-complex molecular featrues and similarities with some human prion diseases

A.G. Biacabe, J.G. Jacobs, A. Bencsik, J.P.M. Langeveld, T.G.M. Baron

    Research output: Contribution to journalArticleAcademicpeer-review

    62 Citations (Scopus)

    Abstract

    We previously reported that some cattle affected by bovine spongiform encephalopathy (BSE) showed distinct molecular features of the protease-resistant prion protein (PrPres) in Western blot, with a 1-2 kDa higher apparent molecular mass of the unglycosylated PrPres associated with labelling by antibodies against the 86-107 region of the bovine PrP protein (H-type BSE). By Western blot analyses of PrPres, we now showed that the essential features initially described in cattle were observed with a panel of different antibodies and were maintained after transmission of the disease in C57Bl/6 mice. In addition, antibodies against the C-terminal region of PrP revealed a second, more C-terminally cleaved, form of PrPres (PrPres #2), which, in unglycosylated form, migrated as a approximate to 14 kDa fragment. Furthermore, a PrPres fragment of approximate to 7 kDa, which was not labelled by C-terminus-specific antibodies and was thus presumed to be a product of cleavage at both N- and C-terminal sides of PrP protein, was also detected. Both PrPres #2 and approximate to 7 kDa PrPres were detected in cattle and in C57Bl/6 infected mice. These complex molecular features are reminiscent of findings reported in human prion diseases. This raises questions regarding the respective origins and pathogenic mechanisms in prion diseases of animals and humans.
    Original languageEnglish
    Pages (from-to)61-68
    Number of pages8
    JournalPrion
    Volume1
    Issue number1
    DOIs
    Publication statusPublished - 2007

    Keywords

    • straussler-scheinker-disease
    • creutzfeldt-jakob-disease
    • monoclonal-antibodies
    • atypical scrapie
    • strain variation
    • sheep scrapie
    • protein
    • mice
    • prp
    • bse

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