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 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.
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Genecenter Munich, Max von Pettenkofer-Institute for Virology, Ludwig-Maximilians-University of Munich, Germany.
Spielhaupter
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Schätzl
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D000483:Alleles; D000595:Amino Acid Sequence; D000818:Animals; D006801:Humans; D007900:Lemur / Q000235:genetics*; D008969:Molecular Sequence Data; D011328:Prions / Q000235:genetics*; D017386:Sequence Homology, Amino Acid
D011328:Prions