Gilch, S.; Spielhaupter, C.; Schätzl, H. M.
Shortest known prion protein allele in highly BSE-susceptible lemurs.
In: Biological Chemistry, Vol. 381, No. 5-6: pp. 521-523
We describe the shortest prion protein allele known to date. Surprisingly, it is found as a polymorphism exactly in a species (prosimian lemurs) which seems highly susceptible to oral infection with BSE-derived prions. The truncation of the prion protein we found raises several questions. First, is the truncated octarepeat structure we describe, consisting of two octarepeats, still functional in copper binding? A second question is whether this truncation is related to the remarkable oral infectibility of lemurs with BSE-derived prions. And finally, one could argue that this genotype alone might favour development of a prion disease, even in the absence of exogenous infection.