NR AWAZ
AU Allix,S.; Ouidja,M.O.; Nguyen,C.H.; El Hachimi,H.K.; Couquet,C.; Deslys,J.P.; Brugere,H.; Brugere-Picoux,J.; Adjou,K.T.
TI Purkinje-cells degeneration in the cerebellum of sheep and goats naturally affected with scrapie
QU International Conference - Prion 2006: Strategies, advances and trends towards protection of society - 3.10.-6.10.2006, Torino, Italy, Lingotto Conference Centre - Poster sessions PA-02
PT Konferenz-Poster
AB The cellular prion protein plays an important role in the pathogenesis of transmissible spongiform encephalopathies (TSEs) such natural scrapie in sheep and goats. Purkinje cells are some of the largest neurons in the cerebellum, with intricately elaborate dendritic trees, characterized by a large number of spines. These send inhibitory projections to the deep cerebellar nuclei, and constitute the sole output of all motor coordination in the cerebellar cortex. The loss of Purkinje cells lead to motor disorders and ataxia. The purpose of this study was to evaluate qualitatively and quantitatively the degenerence of Purkinje cells in the cerebellum of small ruminants affected with natural scrapie. Six brains of Texel ewes and six brains of Alpine goats showing clinical signs of scrapie were examined in the cerebellum for the accumulation of PrPsc and Calbindin-28k protein, a "specific" marker of Purkinje cells. All the animals were confirmed as positive for PrPsc by Bio-Rad rapid test and/or immunohistochemistry. Three ewes (ARR/ARR) and three goats without neurological diseases were used as negative controls. Immunostaining of Calbindin-28k and PrP were performed using respectively a polyclonal anti-Calbindin-28k antibody (Swant, Switzerland) and a monoclonal anti-PrP antibody (8G8, CEA Saclay, France). Our results showed an important loss of dendritic trees of Purkinje cells in the molecular layer and a cell body degeneration in the cellular layer in scrapie animals. The decrease of Purkinje cells number in the cerebellum was estimated at 56% in sheep and 28% in goats comparatively to healthy controls. This major loss of Purkinje cells is correlated with the amounts of PrPsc evidenced by doublelabelling. In conclusion, our results showed an important loss of Purkinje cells in the cerebellum of small ruminants naturally affected with scrapie and this degeneration could explain in part the motor disorders and ataxia usually observed in TSEs.
AD S. Allix, C.H. Nguyen, H. Brugère, J. Brugère-Picoux: Ecole Nationale Vétérinaire d'Alfort, 7 avenue du général de Gaulle, 94704 Maisons-Alfort, France; M.O. Ouidja, K.T. Adjou: Ecole Nationale Vétérinaire d'Alfort, 7 avenue du général de Gaulle, 94704 Maisons-Alfort, France und CEA, GID/TIP/DSV/DRM, 18 route du Panorama, 92 265 Fontenay aux Roses Cedex, France; H.K. El Hachimi: EPHE/INSERM U106, 47 boulevard de l'Hôpital, 75 013 Paris, France; C. Couquet: LDV, avenue du Professeur de Léobardy, 87 000 Limoges, France
SP englisch
PO Italien