How to cite this article: Vet. Res. (2009) 40:48
DOI: 10.1051/vetres/2009031

State-of-the-art review of goat TSE in the European Union, with special emphasis on PRNP genetics and epidemiology

Gabriele Vaccari¹, Cynthia H. Panagiotidis², Cristina Acin³, Simone Peletto⁴, Francis Barillet⁵, Pierluigi Acutis⁴, Alex Bossers⁶, Jan Langeveld⁶, Lucien Van Keulen⁶, Theodoros Sklaviadis², Juan J. Badiola³, Olivier Andreéoletti⁵, Martin H. Groschup⁷, Umberto Agrimi¹, James Foster⁸ and Wilfred Goldmann<sup>8

1</sup>  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
²  Aristotle University of Thessaloniki, School of Health Sciences, Department of Pharmaceutical Sciences, Laboratory of Pharmacology, Thessaloniki 540 06, Greece
³  Centro Nacional de Referencia de las EET en España, Facultad de Veterinaria, Universidad de Zaragoza, C/ Miguel Servet 177, 50013 Zaragoza, Spain
⁴  Centro di Referenza per le Encefalopatie Animali, Istituto Zooprofilattico Sperimentale del Piemonte, Liguria e Valle d'Aosta, Via Bologna 148, 10154 Turin, Italy
⁵  UMR INRA ENVT 1225 Interactions Hôtes Agents Pathogènes, ENVT, 23 Chemin des Capelles, 31076 Toulouse, France
⁶  Central Veterinary Institute of Wageningen UR (CVI), Department of Bacteriology and TSEs, PO Box 65, 8200 AB Lelystad, The Netherlands
⁷  Institute for Novel and Emerging Infectious Diseases at the Friedrich-Loeffler-Institut, Boddenblick 5a, 17493 Greifswald-Insel Riems, Germany
⁸  Roslin Institute and R(D)SVS University of Edinburgh, Roslin, Midlothian, EH25 9PS, United Kingdom

Received 27 November 2008; accepted 22 May 2009; published online 28 May 2009

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.

Key words: transmissible spongiform encephalopathy / prion protein / genetics / goat / scrapie

Corresponding author: wilfred.goldmann@roslin.ed.ac.uk

© INRA, EDP Sciences 2009