Transmissible spongiform encephalopathies

The team of researchers of he Centre has been working for several years in several lines that have had as objective the improvement of the knowledge of different aspects of the transmissible spongiform encephalopathies:

  • Epidemiological studies with the aim of having realistic information on the incidence and prevalence of TSEs in Spain in order to support the development of surveillance and control plans. All these works have been financed by means of European, national or he Autonomous Community of Aragon projects, agreements or contracts (The establishment of a European network for the surveillance of Ruminant TSE and the standardisation and harmonisation of the process and criteria for the identification of suspect cases, FAIR J-CT98-7021; European Concerted Action for the setting up of multicentric epidemiological databases and biological sample banks for small ruminant Scrapie, FAIR J-CT97-6056. Agreement MAPA-UZ, 1996-2015; Agreement Department of Agriculture and Food of Aragon-UZ, 2000-2015).
  • With regard to diagnosis, molecular characterisation of BSE cases that cannot be confirmed by routine diagnostic techniques has been further developed (Agl2006-08467 Molecular and phenotypic characterisation of the causative agent in inconclusive cases of animal transmissible spongiform encephalopathies).
  • Studies related to pathogenesis have also been carried out, analysing histological lesions of nervous tissue and characterising the expression of the PrPsc protein as a response to different infection routes in a murine model developed with transgenic mice (Role of PrP in prion spread and establishment of central nervous system infection, FAIR J-CT98-6022; Detection of preclinical cases of Scrapie: study of the interaction between PrPsc and the lymphoid system and other tissues of a non-nervous nature, UZ2002-BIO-06; Distribution of PrPsc in sheep and goats from scrapie affected flocks in the C.A. de Aragón: Neuropathological profiles, detection of preclinical cases in lymphoid organs and new diagnostic tests; IBE2005- BIO-03; Trans-Pyrenean cooperation in the safety of food of ovine and caprine origin EFA85/08 COTSA) and assessing the effect of different metals administered in the animal’s diet on the development of a spongiform encephalopathy in a mouse model, as well as the mechanisms of transmission (Studies on the mechanisms of transmission of sheep scrapie under natural conditions PM050/2006; Trans-Pyrenean cooperation on food safety in sheep and goat origin EFA85/08 COTSA.) Other studies have aimed to extend the results of the previous ones (European network for surveillance and control of TSE in small ruminants (with emphasis on epidemiology, pathology and diagnostic tests, QRLT-2001-00959), as well as to assess the effect of oxydative stress on disease susceptibility and resistance (Investigating the role of oxydative stress or diet on prion disease susceptibility, QLRT-2001-01871). Other work has been directed towards the search for new diagnostic markers and therapeutic targets (Definition of diagnostic markers and therapeutic targets in animal and human prion diseases, EET2001-4844-C03-01), or the study of the mechanisms of neuroinvasion and neurodegeneration in prion diseases (Molecular mechanisms of scrapie-associated neuropathology: Analysis of neuronal apoptosis and expression profiles, EET2003/ 09890).
  • Several studies have been conducted on the genetic aspects of these diseases (Transmissible Spongiform Encephalopathies (TSEs): Analysis of the PrP gene in sheep, typing and differentiation of prion strains, TSE2001-4033; Generation of a possible strategy for the prevention of transmissible spongiform encephalopathies in goats, PM024; Epidemiological and genetic study of scrapie in sheep and goats in Morocco, AECI-P/03; Study of the PRNP gene in Aragonese goat breeds. Implications on the susceptibility or genetic resistance to scrapie disease and bovine spongiform encephalopathy; IBE2005-BIO-01) Alterations in gene expression in sheep and goats infected by different prion strains; AGL2007-63700/GAN). Also in 2006, a European project was awarded under the 6th Framework Programme that has collaborated in extending this study of goat resistance or susceptibility, delving into other topics such as pathogenesis, infectivity and diagnosis (GoatBSE: proposal for improvement of goat TSE discriminative diagnosis and susceptibility based assessment of bse infectivity in goat milk and meat 2006-2010). The results obtained in this project have allowed the identification of reference codons that can be selected in the goat species for their resistance to scrapie. Thus, thanks to the concession of a new project of the EMIDA network (Towards breeding of goats for genetically determined TSEs resistance) and the Ministry of Economy and Competitiveness (Effect of the PRNP gene genotype in the goat species on the susceptibility to BSE), the aim is to define the codons to be selected at a global level for the breeding of goats resistant to transmissible spongiform encephalopathies.
  • It should be noted that the Centre has been considered as a Consolidated Group and a Group of Research Excellence by the Government of Aragon (Research Group on prion diseases and animal lentiviruses. Consolidated Group DGA 2005. a25 and successive ones until the present time) and participates or has participated in several research networks, both at national level (Research network of neurological diseases. Red CIEN of the Instituto Carlos III, Redes Temáticas de Investigación Cooperativa, Ministerio de Sanidad y Consumo, Red Nacional de Priones ) and internationally (European network for surveillance and control of TSE in small-ruminants, with emphasis on epidemiology, pathology and diagnostic tests, Setting up of multicentric epidemiological data bases and biological samples banks for small ruminant scrapie, Red Europea transpirenaica en seguridad alimentaria, Network of Excellence: Prevention, control and management of prion diseases”, FOOD-2004-506579). The last of these includes the most important research groups in the EU, with the Zaragoza centre being the leader of one of the network’s priority lines and one of the European tissue banks at the service of the international scientific community. This places the Centre in a privileged position as it has rapid access to all types of information related to the subject, as well as the possibility of participation and interaction with different groups dedicated to research into different aspects of TSEs.
  • The Centre also participates or has actively participated in R+D+i contracts financed by companies for the validation of diagnostic tests (Evaluation of the CDI test for the diagnosis of transmissible spongiform encephalopathies, InPro Differentiation and distribution of PrPsc in tissue of ovine breeds in Spain using Prionics Check Diagnostic Test Systems and reagents, Prionics AG), in the development of new diagnostic tests (Exchange of sheep samples and collaboration on test development for scrapie in small ruminants, Prionics AG) and in the improvement of food safety (Trans-Pyrenean European Network on Food Safety), Scrapie disease-free sheep certification programme, Mercadona S.A.; Optimisation of lamb production to offer greater health guarantees, product quality and animal welfare. Design of preventive and prophylactic protocols during the rearing of lambs by Carnes Oviaragón S.C.L, Carnes Oviaragón S.C.L; Protocol for the detection of the central nervous system in meat from the head of cattle over 12 months old, destined for human consumption; Trans-Pyrenean cooperation in the safety of food of ovine and caprine origin (EFA85/08 COTSA).