title: State-of-the-art review of goat TSE in the European Union, with special emphasis on PRNP genetics and epidemiology.
abstract: Scrapie is a fatal, neurodegenerative disease of sheep and goats. It is also the earliest known member in the family of diseases classified as transmissible spongiform encephalopathies (TSE) or prion diseases, which includes Creutzfeldt-Jakob disease in humans, bovine spongiform encephalopathy (BSE), and chronic wasting disease in cervids. The recent revelation of naturally occurring BSE in a goat has brought the issue of TSE in goats to the attention of the public. In contrast to scrapie, BSE presents a proven risk to humans. The risk of goat BSE, however, is difficult to evaluate, as our knowledge of TSE in goats is limited. Natural caprine scrapie has been discovered throughout Europe, with reported cases generally being greatest in countries with the highest goat populations. As with sheep scrapie, susceptibility and incubation period duration of goat scrapie are most likely controlled by the prion protein (PrP) gene (PRNP). Like the PRNP of sheep, the caprine PRNP shows significantly greater variability than that of cattle and humans. Although PRNP variability in goats differs from that observed in sheep, the two species share several identical alleles. Moreover, while the ARR allele associated with enhancing resistance in sheep is not present in the goat PRNP, there is evidence for the existence of other PrP variants related to resistance. This review presents the current knowledge of the epidemiology of caprine scrapie within the major European goat populations, and compiles the current data on genetic variability of PRNP.
authors.affiliation: Department of Food Safety and Veterinary Public Health, Unit of Transmissible Spongiform Encephalopathies and Emerging Infectious Diseases of Animals, Istituto Superiore di Sanità, Viale Regina Elena 299, 00161, Rome, Italy.
authors.forename: Gabriele
authors.initials: G
authors.lastname: Vaccari
authors.forename: Cynthia H
authors.initials: CH
authors.lastname: Panagiotidis
authors.forename: Cristina
authors.initials: C
authors.lastname: Acin
authors.forename: Simone
authors.initials: S
authors.lastname: Peletto
authors.forename: Francis
authors.initials: F
authors.lastname: Barillet
authors.forename: Pierluigi
authors.initials: P
authors.lastname: Acutis
authors.forename: Alex
authors.initials: A
authors.lastname: Bossers
authors.forename: Jan
authors.initials: J
authors.lastname: Langeveld
authors.forename: Lucien
authors.initials: L
authors.lastname: van Keulen
authors.forename: Theodoros
authors.initials: T
authors.lastname: Sklaviadis
authors.forename: Juan J
authors.initials: JJ
authors.lastname: Badiola
authors.forename: Olivier
authors.initials: O
authors.lastname: Andreéoletti
authors.forename: Martin H
authors.initials: MH
authors.lastname: Groschup
authors.forename: Umberto
authors.initials: U
authors.lastname: Agrimi
authors.forename: James
authors.initials: J
authors.lastname: Foster
authors.forename: Wilfred
authors.initials: W
authors.lastname: Goldmann
mesh_terms: D000818:Animals; D005060:Europe / Q000453:epidemiology; D015511:Goat Diseases / Q000453:epidemiology*; D006041:Goats; D011328:Prions / Q000235:genetics*; D012608:Scrapie / Q000453:epidemiology*
chemical_list: D011328:Prions