Shortest known prion protein allele in highly BSE-susceptible lemurs

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 tr uncation of the prion protein we found raises several questions. First, is the truncated octarepeat structure we describe, consisting of two octarepea ts, still functional in copper binding? A second question is whether this t runcation is related to the remarkable oral infectibility of lemurs with BS E-derived prions. And finally, one could argue that this genotype alone mig ht favour development of a prion disease, even in the absence of exogenous infection.