1887

Abstract

Prion diseases in ruminants, especially sheep scrapie, cannot be fully explained by genetics, suggesting the influence of a second modulator gene. The gene is a good candidate for this role. The gene encodes the shadoo protein (Sho) which has homology to the gene encoding prion protein (PrP). Murine Sho has a similar neuroprotective activity to PrP and gene variants are associated with human prion disease susceptibility. gene sequences were obtained from 14 species in the orders Artiodactyla and Rodentia. We report here the sequences of more than 20 different Sho proteins that have arisen due to single amino acid substitutions and amino acid deletions or insertions. All Sho sequences contained an alanine-rich sequence homologous to a hydrophobic region with amyloidogenic characteristics in PrP. In contrast with PrP, the Sho sequence showed variability in the number of alanine residues.

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2009-10-01
2019-11-12
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