2000
DOI: 10.1515/bc.2000.067
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Shortest Known Prion Protein Allele in Highly BSE-Susceptible Lemurs

Abstract: 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 lemu… Show more

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Cited by 4 publications
(5 citation statements)
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“…Reports that PRNP alleles with two repeats occur in lemurs (Gilch et al 2000) and red squirrel (van Rheede et al 2003) might imply that prion proteins with only two repeats can be functionally viable. It is therefore important that we here demonstrate that the squirrel two-repeat PRNP sequence actually represents a non-expressed retroposed pseudogene.…”
Section: Discussionmentioning
confidence: 99%
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“…Reports that PRNP alleles with two repeats occur in lemurs (Gilch et al 2000) and red squirrel (van Rheede et al 2003) might imply that prion proteins with only two repeats can be functionally viable. It is therefore important that we here demonstrate that the squirrel two-repeat PRNP sequence actually represents a non-expressed retroposed pseudogene.…”
Section: Discussionmentioning
confidence: 99%
“…This not only means that any concerns about consuming squirrel brains (Berger et al 1997; Sekercioglu 2004) can not be related to the expression of a two-repeat PrP, but, more importantly, also raises interesting questions about prion structure, function and evolution. In the case of the reported lemur two-repeat PRNP genomic sequence, it can not be decided from the available data whether it is actually expressed, or whether it shows any further differences with the lemur five-repeat PRNP sequence (Gilch et al 2000). Since the lemur two-repeat PRNP sequence reportedly occurred in two individuals from different genera, it might be expected to be a more common feature in lemurs.…”
Section: Discussionmentioning
confidence: 99%
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“…Deletion of one octareapeat seems to be a protective factor in goats infected with caprine prions (Goldmann et al, 1998). On the other hand, it was shown that lemurs, which carry the shortest octarepeat region known to date (consisting of only two octarepeats), are highly susceptible to BSE prions (Gilch et al, 2000). Rhesus macaques were shown to have 5 octarepeats (Schatzl et al, 1997).…”
Section: Analysis Of the Prion Protein In Primates Reveals A New Polymentioning
confidence: 99%