Marruchella, Giuseppe and Malatesta, Daniela and Petrucci, Luigi and Lalatta-Costerbosa, Giovanna and Clavenzani, Paolo and Chiocchetti, Roberto and Mazzoni, Maurizio and Albanese, Valeria and Agrimi, Umberto and D'Agostino, Claudia and Vaccari, Gabriele and Nonno, Romolo and De Grossi, Luigi and Rosone, Francesca and Giordani, Francesco and Ligios, Ciriaco and Sarli, Giuseppe and Di Guardo, Giovanni (2006) Pathogenetic investigations on the enteric nervous system plexuses of Sarda breed sheep with different PrP genotypes following oral experimental scrapie infection. In: 3. Congresso nazionale A.I.P.Vet.: atti, 11-13 Maggio 2006, Pisa, Italia. Milano, AIPVet. p. 26-27. ISSN 1825-2265. Conference or Workshop Item.
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The enteric nervous system (ENS) plays a key role in sheep scrapie, but no information exists on the cytotypes
which are involved during infection, nor on the damage of such cells.
We investigated the ileal myenteric (MPs) and submucosal plexuses (SMPs) of 32 Sarda breed sheep carrying
different PrP genotypes (ARQ/ARQ, ARQ/AHQ, ARQ/ARR, ARR/ARR), which had been orally dosed with
scrapie at 8 months of age and euthanized at definite time intervals post-infection (p.i.). PrPSc immunoreactivity
(IR), along with neuronal marker Hu C/D, nitric oxide synthase (nNOS), calbindin (CALB), glial fibrillary acidic
protein (GFAP) and synaptophysin IR, were evaluated by immunohistochemistry (IHC) and indirect
immunofluorescence on paraffin sections and wholemount preparations.
Eight clinically-healthy ARQ/ARQ sheep euthanized at 12-24 months p.i., along with 1 ARQ/ARQ (euthanized
at 24 months p.i.) and 3 ARQ/AHQ (euthanized at 35, 36.3 and 39.5 months p.i., respectively) clinically-affected
sheep, showed IHC evidence of PrPSc in both their brain (obex) and ENS, especially in MPs. PrPSc depositino
was compatible with an involvement of enteroglial cells (EGCs) and a dramatic reduction (P value < 0.05) of
CALB-IR neurons was observed in the MPs of 6 ARQ/ARQ animals, as compared to normal healthy controls
and to infected subjects of different PrP genotypes, which also showed an almost exclusive nuclear IR of these
Therefore, EGCs and CALB-IR neurons of ileal ENS plexuses are likely involved in sheep scrapie.
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