Enteroglial and neuronal involvement without apparent neuron loss in ileal enteric nervous system plexuses from scrapie-affected sheep.
The enteric nervous system (ENS) probably plays a dominant role in sheep scrapie pathogenesis, but little is known about the cell types involved. We investigated the ileal myenteric and submucosal plexuses of four naturally and four orally experimentally scrapie-affected ARQ/ARQ Sarda sheep, as well as those of 12 healthy-control Sarda sheep carrying different PrP genotypes. All scrapie-affected animals, euthanized at clinical-disease end stage, showed PrPd deposition within enteric glial cells (EGCs) and calbindin-immunoreactive (CALB-IR) and neuronal nitric oxide synthase (nNOS)-IR neurons. Whole-mount investigations revealed no significant differences between the densities of total, CALB-IR and nNOS-IR neurons in scrapie-affected versus healthy sheep, irrespective of PrP genotype. Our results suggest that EGCs and CALB-IR and nNOS-IR neurons are probably involved in the pathogenesis of natural and oral experimental sheep scrapie. Furthermore, the infectious agent may be less pathogenic towards ENS neurons than it is towards central nervous system neurons.
Marruchella
Giuseppe
G
Department of Comparative Biomedical Sciences, Faculty of Veterinary Medicine, University of Teramo, Teramo, Italy.
Ligios
Ciriaco
C
Istituto Zooprofilattico Sperimentale della Sardegna, Sassari, Italy.
Albanese
Valeria
V
Department of Comparative Biomedical Sciences, Faculty of Veterinary Medicine, University of Teramo, Teramo, Italy.
Cancedda
Maria Giovanna
MG
Istituto Zooprofilattico Sperimentale della Sardegna, Sassari, Italy.
Madau
Laura
L
Istituto Zooprofilattico Sperimentale della Sardegna, Sassari, Italy.
Lalatta-Costerbosa
Giovanna
G
Department of Veterinary Morphophysiology and Animal Productions, Faculty of Veterinary Medicine, University of Bologna, Ozzano Emilia (Bologna), Italy.
Mazzoni
Maurizio
M
Department of Veterinary Morphophysiology and Animal Productions, Faculty of Veterinary Medicine, University of Bologna, Ozzano Emilia (Bologna), Italy.
Clavenzani
Paolo
P
Department of Veterinary Morphophysiology and Animal Productions, Faculty of Veterinary Medicine, University of Bologna, Ozzano Emilia (Bologna), Italy.
Chiocchetti
Roberto
R
Department of Veterinary Morphophysiology and Animal Productions, Faculty of Veterinary Medicine, University of Bologna, Ozzano Emilia (Bologna), Italy.
Sarli
Giuseppe
G
Department of Veterinary Public Health and Animal Pathology, Division of Veterinary Pathology, Faculty of Veterinary Medicine, University of Bologna, Ozzano Emilia (Bologna), Italy.
De Grossi
Luigi
L
Istituto Zooprofilattico Sperimentale delle Regioni Lazio e Toscana, Viterbo, Italy.
Agrimi
Umberto
U
Istituto Superiore di Sanità, Department of Food Safety and Veterinary Public Health, Rome, Italy.
Aguzzi
Adriano
A
Institute of Neuropathology, University Hospital of Zurich, Zurich, Switzerland.
Di Guardo
Giovanni
G
Department of Comparative Biomedical Sciences, Faculty of Veterinary Medicine, University of Teramo, Teramo, Italy.
D000818:Animals; D006801:Humans; D009457:Neuroglia / Q000473:pathology* / Q000821:virology; D009474:Neurons / Q000201:enzymology / Q000473:pathology*; D052248:Nitric Oxide Synthase Type I / Q000032:analysis; D017096:Prion Diseases / Q000473:pathology / Q000635:transmission; D012016:Reference Values; D012608:Scrapie / Q000473:pathology*; D012756:Sheep
D052248:Nitric Oxide Synthase Type I