NR AXRK
AU Liberski,P.P.; Sikorska,B.; Hauw,J.J.; Kopp,N.; Streichenberger,N.; Giraud,P.; Budka,H.; Boellaard,J.W.; Brown,P.
TI Tubulovesicular Structures are a Consistent (and Unexplained) Finding in the Brains of Humans with Prion Diseases
QU International Conference - Prion 2007 (26.-28.9.2007) Edinburgh International Conference Centre, Edinburgh, Scotland, UK - Book of Abstracts: Pathology and Pathogenesis P03.135
IA http://www.prion2007.com/pdf/Prion Book of Abstracts.pdf
PT Konferenz-Poster
AB
Tubulovesicular structures appear to be disease-specific markers for all TSEs, including CJD (Liberski et al., 1990, 2005), GSS (Liberski & Budka, 1994), vCJD, FFI (Liberski et al., 2005), naturally and experimentally induced scrapie (Liberski et al., 1990), and BSE (Liberski et al., 1992). Most recently, virus-like structures closely similar to TVS were observed in cells cultures infected with scrapie and CJD (Manuelidis et al., 2007). Here we report ultrastructural findings in 21 brain biopsy or autopsy specimens from patients with TSEs, and in 18 specimens from patients with other neurodegenerative conditions, including a small coded series examined before the diagnosis was revealed.
In the first (coded) series, TVS were found in all five specimens of CJD and in none of four AD brains. In the second series, TVS were found in 12 of 13 specimens, including the single cases of FFI and vCJD; the only negative specimen was from the case of familial CJD. TVS were also found in all three archival cases of GSS and one autopsy FFI case of the third series, but were not found in any of the 14 non-TSE neurological control specimens.
Morphologically, TVS appeared as clusters of round or tubular 25-35 nm particles with light cores surrounded by darker 'capsules'. When present, these aggregates were rare - approximately 1-2 TVS-containing neuronal processes per specimen (mostly dendrites, rarely, synaptic terminals). The finding of TVS in 20 of 21 cases of human TSE but in none of 18 non-TSE neurological control specimens extends our previous studies and confirms their specificity in both naturally occurring and experimentally induced TSEs. The nature of TVS remains enigmatic and their pathogenetic role remains to be determined, but three observations are worthy of comment: first, the appearance of TVS precedes that of other pathological phenomena, and their number increases through the incubation period in both rodent scrapie and CJD models; second, the 2735 nm size of TVS corresponds well to 27-nm cut-off of ultrafiltration studies for infectivity; and third, TVS are not composed of the abnormal isoform of PrP.
AD P.P. Liberski, B. Sikorska, Medical University Lodz, Chair of Oncology, Molecular Pathology and Neuropathology, Poland; J.-J. Hauw, La Pitie Salpetriere Hôpital, Raymond Escourolle Neuropathological Laboratory, France; N. Kopp, N. Streihenberger, P. Giraud, Hôpital Neurologique et Neuro-chirurgical Pierre Wertheimer, Laboratoire d'Anatomie Pathologique et de Neuropat, France; H. Budka, Medical University of Vienna, Institute of Neurology and Austrian Reference Cent, Austria; J. Boellaard, Retired, Germany; P. Brown, Retired, USA
SP englisch
PO Schottland