NR AURK
AU Kuczius,T.; Grassi,J.; Karch,H.; Groschup,M.H.
TI Phenotypic variability of full length prions in different species
QU TSE-Forum, 6. Kongress - Nationale TSE-Forschungsplattform, Greifswald 26.6.-28.6.2006, Poster: Struktur und molekulare Mechanismen MOL-08
PT Konferenz-Poster
AB
Transmissible spongiform encephalopathies (TSEs) or prion diseases are fatal neurodegenerative disorders which affect humans and many species of animals. TSEs include scrapie in sheep and goats and bovine spongiform encephalopathy (BSE) in cattle. Human prion diseases comprise sporadic Creutzfeldt-Jakob disease (sCJD), genetic forms as familiar CJD and infectious forms as the new variant (vCJD) which has been linked to BSE.
The infectious agent is composed of an abnormal, partially protease resistant isoform (PrPsc) of a host encoded cellular prion protein (PrPc) that plays an essential role in the development of the disease. PrPsc proteins are characterized by size and glycoform patterns using molecular typing techniques. Recently, multiple patterns have been identified coexisting in CJD patients. The conversion to PrPsc and the efficiency of accumulation may be influenced by expression of different PrPc types. In this study we analysed the glycoform patterns of PrPc derived from human, sheep, cattle and mouse and compared them with each other. Using a panel of antibodies recognizing various epitopes within the PrP sequence we found two distinct types independently on the species. High signal intensities were detected for the non glycosylated isoform when PrPc was determined by antibodies recognizing the carboxyl terminal region as well as for the diglycosylated isoform by antibodies binding to the amino terminal region. These data demonstrate variations of antibody binding resulting in distinct and distinguishable banding patterns and strengthens the importance of the antibody choice for characterization.
AD Thorsten Kuczius, Helge Karch, Institut für Hygiene, Universitätsklinikum Münster; Jacques Grassi, CEA, Service de Pharmacologie et d'Immunologie, Saclay; Martin H. Groschup, Friedrich-Loeffler-Institut, Institut für neue und neuartige Tierseuchenerreger, Insel Riems
SP englisch
PO Deutschland
OR Tagungsband