Abstract

BackgroundSince 2002, active surveillance programmes have detected numerous atypical scrapie (AS) and classical scrapie cases (CS) in French sheep with almost all the PrP genotypes. The aim of this study was 1) to quantify the genetic risk of AS in French sheep and to compare it with the risk of CS, 2) to quantify the risk of AS associated with the increase of the ARR allele frequency as a result of the current genetic breeding programme against CS.MethodsWe obtained genotypes at codons 136, 141, 154 and 171 of the PRNP gene for representative samples of 248 AS and 245 CS cases. We used a random sample of 3,317 scrapie negative animals genotyped at codons 136, 154 and 171 and we made inferences on the position 141 by multiple imputations, using external data. To estimate the risk associated with PrP genotypes, we fitted multivariate logistic regression models and we estimated the prevalence of AS for the different genotypes. Then, we used the risk of AS estimated for the ALRR-ALRR genotype to analyse the risk of detecting an AS case in a flock homogenous for this genotype.ResultsGenotypes most at risk for AS were those including an AFRQ or ALHQ allele while genotypes including a VLRQ allele were less commonly associated with AS. Compared to ALRQ-ALRQ, the ALRR-ALRR genotype was significantly at risk for AS and was very significantly protective for CS. The prevalence of AS among ALRR-ALRR animals was 0.6‰ and was not different from the prevalence in the general population.ConclusionIn conclusion, further selection of ALRR-ALRR animals will not result in an overall increase of AS prevalence in the French sheep population although this genotype is clearly susceptible to AS. However the probability of detecting AS cases in flocks participating in genetic breeding programme against CS should be considered.

Highlights

  • Since 2002, active surveillance programmes have detected numerous atypical scrapie (AS) and classical scrapie cases (CS) in French sheep with almost all the PrP genotypes

  • Bovine spongiform encephalopathy (BSE) is a zoonotic transmissible spongiform encephalopathy (TSE), which may have spread to sheep and goat populations

  • The ALHQ-ALHQ, AFRQ-ALHQ and AFRQAFRQ genotypes were associated with the highest risks of atypical form of scrapie (AS) compared to ALRQ-ALRQ

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Summary

Introduction

Since 2002, active surveillance programmes have detected numerous atypical scrapie (AS) and classical scrapie cases (CS) in French sheep with almost all the PrP genotypes. Bovine spongiform encephalopathy (BSE) is a zoonotic transmissible spongiform encephalopathy (TSE), which may have spread to sheep and goat populations This situation has prompted European countries to organise control and surveillance of TSEs in small ruminants in which BSE cannot be clinically distinguished from scrapie. The main alleles defined by these three codons can be classified by increasing risk as follows ARR < AHQ < ARQ ≈ ARH < VRQ [2]. This knowledge has made it possible to implement eradication programmes throughout Europe based on positive selection of the most resistant allele (ARR) and negative selection of the most susceptible one (VRQ) [2,3].

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