Transmissible spongiform encephalopathies (TSE) are fatal neurodegenerative diseases of a number of mammalian species. TSE are characterised by the accumulation of an abnormal form of a host-encoded protein, PrP, in the central nervous system of affected individuals. In sheep, mouse and human, these diseases are genetically controlled and a large part of the natural susceptibility to TSE depends on inherited alleles of the Prnp gene encoding PrP protein. However, all individuals with similar PrP alleles may not contract the disease and if they do, they may have very different incubation periods, suggesting that other environmental and genetic factors influence susceptibility to TSE. This paper presents and compares several studies searching QTL, other than the PrP gene, influencing susceptibility to TSE in mice and sheep. Firstly, mouse inbred lines with defined Prnp alleles and different incubation periods offer the opportunity to look for genes influencing the outcome of the disease using the QTL methodology. In that way, several research groups identified genetic loci involved in mouse susceptibility to TSE (Stephenson et al., 2000 ; Manolakou et al., 2001 ; Lloyd et al., 2001 ; Moreno et al., in preparation). Secondly, locations of a few QTLs found in the mouse were used to determine homologous regions on the sheep genome. In these regions, microsatellite markers were typed on two half-sib families of ARQ-VRQ sheep (susceptible PrP genotype) to search QTL. The first results are presented.
Proceedings of the World Congress on Genetics Applied to Livestock Production, Volume 2002. Session 13, , 13.21, 2002
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