Abstract
The presence of lysine (K) at codon 222 has been associated with resistance to classical scrapie in goats, but few scrapie cases have been identified in 222Q/K animals. To investigate the contribution of the 222K variant to PrPres formation in natural and experimental Q/K scrapie cases, we applied an immunoblotting method based on the use of two different monoclonal antibodies, F99/97.6.1 and SAF84, chosen for their different affinities to 222K and 222Q PrP variants. Our finding that PrPres seems to be formed nearly totally by the 222Q variant provides evidence that the 222K PrP variant confers resistance to conversion to PrPres formation and reinforces the view that this mutation has a protective role against classical scrapie in goats.
Highlights
Scrapie, an infectious neurodegenerative disease within the group of transmissible spongiform encefalopathies (TSEs), known as prion diseases, affects sheep and goats
Few natural scrapie cases have been identified in 222Q/K goats in Greece and France, and intracerebral experimental transmission was successful after long incubation periods and with low attack rates in both heterozygous (222Q/K) and homozygous (222K/K) goats [11,12,13]
Carrying this polymorphism does not fully protect against classical scrapie, the low incidence of natural scrapie in goats carrying these PrP variants suggests a low tendency of PrP with lysine at position 222 to convert into the pathological isoform (PrPres)
Summary
Maria Mazza,1,* Chiara Guglielmetti, Francesco Ingravalle, Sonia Brusadore, Jan P. Ekateriniadou, Olivier Andreoletti, Cristina Casalone and Pier Luigi Acutis
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