State-of-the-art review of goat TSE in the European Union, with special emphasis on PRNP genetics and epidemiology.
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.
Vaccari
Gabriele
G
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.
Panagiotidis
Cynthia H
CH
Acin
Cristina
C
Peletto
Simone
S
Barillet
Francis
F
Acutis
Pierluigi
P
Bossers
Alex
A
Langeveld
Jan
J
van Keulen
Lucien
L
Sklaviadis
Theodoros
T
Badiola
Juan J
JJ
Andreéoletti
Olivier
O
Groschup
Martin H
MH
Agrimi
Umberto
U
Foster
James
J
Goldmann
Wilfred
W
D000818:Animals; D005060:Europe / Q000453:epidemiology; D015511:Goat Diseases / Q000453:epidemiology*; D006041:Goats; D011328:Prions / Q000235:genetics*; D012608:Scrapie / Q000453:epidemiology*
D011328:Prions