Classical and Atypical Scrapie in Sheep and Goats. Review on the Etiology, Genetic Factors, Pathogenesis, Diagnosis, and Control Measures of Both Diseases.

Cristina Acín,Diego Sola,Belén Marín,Antonia Vargas,Mirta García,Juan José Badiola,Hicham Filali,Eva Monleón,Marina Betancor,Bernardino Moreno,Rosa Bolea,Isabel M Guijarro,Marta Monzón

doi:10.3390/ani11030691

Cristina Acín, Diego Sola + Show 11 more

Open Access

https://doi.org/10.3390/ani11030691

Copy DOI

Abstract

Prion diseases, such as scrapie, are neurodegenerative diseases with a fatal outcome, caused by a conformational change of the cellular prion protein (PrPC), originating with the pathogenic form (PrPSc). Classical scrapie in small ruminants is the paradigm of prion diseases, as it was the first transmissible spongiform encephalopathy (TSE) described and is the most studied. It is necessary to understand the etiological properties, the relevance of the transmission pathways, the infectivity of the tissues, and how we can improve the detection of the prion protein to encourage detection of the disease. The aim of this review is to perform an overview of classical and atypical scrapie disease in sheep and goats, detailing those special issues of the disease, such as genetic factors, diagnostic procedures, and surveillance approaches carried out in the European Union with the objective of controlling the dissemination of scrapie disease.

Highlights

Transmissible spongiform encephalopathies (TSE) or prion diseases are a group of neurodegenerative diseases, of fatal outcome, caused by a conformational change of the cellular prion protein (PrPC ), giving rise to the pathogenic form (PrPSc )
Prion diseases affect humans as well as domestic and wild animals, and in total, eighteen different diseases have been described, nine in animals and nine in humans. They are characterized by astrocyte hyperplasia and hypertrophy, spongiform degeneration, and the accumulation of PrPSc, in the central nervous system (CNS)
This review aims to give an overview of classical and atypical scrapie in sheep and goats, with special emphasis on the genetic factors, diagnostic procedures, and surveillance approaches in the European Union (EU) to control the spread of these diseases

Summary

Introduction

Transmissible spongiform encephalopathies (TSE) or prion diseases are a group of neurodegenerative diseases, of fatal outcome, caused by a conformational change of the cellular prion protein (PrPC ), giving rise to the pathogenic form (PrPSc ). Prion diseases affect humans as well as domestic and wild animals, and in total, eighteen different diseases have been described (see Table 1), nine in animals and nine in humans. They are characterized by astrocyte hyperplasia and hypertrophy, spongiform degeneration, and the accumulation of PrPSc , in the central nervous system (CNS). The first reliable records on TSE data are from the 18th century where classical scrapie was described [1] This disease, which affects sheep and goats, is characterized by a progressive chronic ataxia that leads to a lack of coordination, intense scratching, and changes in behavior.

Objectives

Methods

Conclusion