NR AXYY
AU Wrede,A.; Beekes,M.; Thomzig,A.; Schulz-Schaeffer,W.J.
TI Subcellular Localization of PrPsc in Muscle Fibres
QU International Conference - Prion 2007 (26.-28.9.2007) Edinburgh International Conference Centre, Edinburgh, Scotland, UK - Book of Abstracts: Pathology and Pathogenesis P03.12
IA http://www.prion2007.com/pdf/Prion Book of Abstracts.pdf
PT Konferenz-Poster
AB To elucidate aspects of prion spread throughout the body we used the well established model of challenging hamsters orally with 263K-scrapie strain prion protein. This model shows centripetal as well as centrifugal propagation of pathological prion protein (PrPsc) and can be regarded as an exemplary model for naturally occurring transmissible spongiform encephalopathies (TSE's). When PrPsc has reached the central nervous system after oral challenge, it undergoes a centrifugal spread to peripheral organs, amongst others PrPsc is entering muscle tissue. PrPsc reaches muscle fibres via small innervating nerve fibres and is found at the neuromuscular junction (NMJ) within the presynaptic nerve endings. This stage is presumably followed by a second stage, in which PrPsc can be detected at the postsynaptic zone of the NMJ within muscle fibres. Additionally we could demonstrate fibres showing a disseminated distribution across at least expanded areas of muscle fibres reminiscent of a distribution pattern following structures of the plasma membrane and its invaginations, the T-tubules. This staining pattern led us to the hypothesis that there is probably a muscular phenotype within certain animals suffering from a prion-disease. In order to approach this hypothesis we applied classical techniques of enzymology to study structural and enzymatical alterations within affected muscle fibres. Additionally we used double immunohistochemistry to find out in which subcellular compartiment PrPsc is located. To verify and to specify these results we did electron microscopic investigations. Intending to define a possible morphological phenotype we want to present data from these enzymatic and immunohistochemical staining patterns as well as electron microscopic evaluations showing characteristic changes within infected muscle fibres.
AD A. Wrede, W.J. Schulz-Schaeffer, Georg-August-University of Göttingen, Dept. of Neuropathology, Germany; M. Beekes, A. Thomzig, P24 - Transmissible Spongiforme Enzephalopathien, Robert-Koch-Institut, Germany
SP englisch
PO Schottland