title: Enteroglial and neuronal involvement without apparent neuron loss in ileal enteric nervous system plexuses from scrapie-affected sheep.
abstract: 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.
authors.affiliation: Department of Comparative Biomedical Sciences, Faculty of Veterinary Medicine, University of Teramo, Teramo, Italy.
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Marruchella
authors.affiliation: Istituto Zooprofilattico Sperimentale della Sardegna, Sassari, Italy.
authors.forename: Ciriaco
authors.initials: C
authors.lastname: Ligios
authors.affiliation: Department of Comparative Biomedical Sciences, Faculty of Veterinary Medicine, University of Teramo, Teramo, Italy.
authors.forename: Valeria
authors.initials: V
authors.lastname: Albanese
authors.affiliation: Istituto Zooprofilattico Sperimentale della Sardegna, Sassari, Italy.
authors.forename: Maria Giovanna
authors.initials: MG
authors.lastname: Cancedda
authors.affiliation: Istituto Zooprofilattico Sperimentale della Sardegna, Sassari, Italy.
authors.forename: Laura
authors.initials: L
authors.lastname: Madau
authors.affiliation: Department of Veterinary Morphophysiology and Animal Productions, Faculty of Veterinary Medicine, University of Bologna, Ozzano Emilia (Bologna), Italy.
authors.forename: Giovanna
authors.initials: G
authors.lastname: Lalatta-Costerbosa
authors.affiliation: Department of Veterinary Morphophysiology and Animal Productions, Faculty of Veterinary Medicine, University of Bologna, Ozzano Emilia (Bologna), Italy.
authors.forename: Maurizio
authors.initials: M
authors.lastname: Mazzoni
authors.affiliation: Department of Veterinary Morphophysiology and Animal Productions, Faculty of Veterinary Medicine, University of Bologna, Ozzano Emilia (Bologna), Italy.
authors.forename: Paolo
authors.initials: P
authors.lastname: Clavenzani
authors.affiliation: Department of Veterinary Morphophysiology and Animal Productions, Faculty of Veterinary Medicine, University of Bologna, Ozzano Emilia (Bologna), Italy.
authors.forename: Roberto
authors.initials: R
authors.lastname: Chiocchetti
authors.affiliation: Department of Veterinary Public Health and Animal Pathology, Division of Veterinary Pathology, Faculty of Veterinary Medicine, University of Bologna, Ozzano Emilia (Bologna), Italy.
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Sarli
authors.affiliation: Istituto Zooprofilattico Sperimentale delle Regioni Lazio e Toscana, Viterbo, Italy.
authors.forename: Luigi
authors.initials: L
authors.lastname: De Grossi
authors.affiliation: Istituto Superiore di Sanità, Department of Food Safety and Veterinary Public Health, Rome, Italy.
authors.forename: Umberto
authors.initials: U
authors.lastname: Agrimi
authors.affiliation: Institute of Neuropathology, University Hospital of Zurich, Zurich, Switzerland.
authors.forename: Adriano
authors.initials: A
authors.lastname: Aguzzi
authors.affiliation: Department of Comparative Biomedical Sciences, Faculty of Veterinary Medicine, University of Teramo, Teramo, Italy.
authors.forename: Giovanni
authors.initials: G
authors.lastname: Di Guardo
mesh_terms: 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
chemical_list: D052248:Nitric Oxide Synthase Type I