NR AXMR
AU Füzi,M.
TI Is Natural Prion Disease in Sheep, Goat and Cervids Triggered by a Microbial Protein?
QU International Conference - Prion 2007 (26.-28.9.2007) Edinburgh International Conference Centre, Edinburgh, Scotland, UK - Book of Abstracts: Epidemiology, Risk Assessment and Transmission P04.18
IA http://www.prion2007.com/pdf/Prion Book of Abstracts.pdf
PT Konferenz-Poster
AB
Though there is overwhelming evidence for a conformationally altered form of PrP being the causative agent of prion diseases the reason why scrapie of sheep and goat and chronic wasting disease (CWD) of cervids - in contrast to prion disease in any other species - spread by contact remains elusive. Observations made by researchers investigating the transmissibility of scrapie that only sheep and goat with natural scrapie transmit prion disease horizontally suggest that a vector with a limited host range is responsible for "contact transmission". Since many microbes are well-known to have a limited host range and reside in the alimentary tract - where natural infection with scrapie and CWD initiates - a microorganism would make a promising candidate for the vector. A recent report by Mathiason et al. /Science 2006, 314, 133-36/ that natural CWD is transmissible with saliva only if it is administered perorally but not if given by injection provides additional evidence. When delivered perorally the microbe should be capable of establishing long-term colonization of the alimentary tract and one of its proteins might gain access to PrPc to alter its conformation. In contrast, if the microbe is introduced parenterally it will soon be destroyed by host defenses.
There could be many additional degenerative diseases developing subsequent to infection with microbes showing similar pathomechanisms. E. g. rheumatic fever caused by the M protein of Streptococcus pyogenes displaying an approximately 40 per cent homology to cardiac myosin may develop after extended streptococcal disease of the upper respiratory tract or of the skin but never following short-term septicemia. Moreover, streptococcal M protein has multiple serotypes, displaying slightly diverse amino acid sequences and eliciting rheumatic fever with distinct efficiencies. The microbial protein triggering scrapie and CWD might also command several serotypes that could account for the existence of the strains of prion.
AD M. Fuzi, National Center for Epidemiology, Hungary
SP englisch
PO Schottland
EA pdf-Datei und Poster (Postertitel: Are natural scrapie and chronic wasting disease (CWD) triggered by a microbial protein capable of adhering to normal prion protein (PrPc) and altering its conformation?)