Abstract：
:It is well established that the conversion of PrP(C) to PrP(Sc) is the key event in prion disease biology. In addition, several lines of evidence suggest that glycosaminoglycans (GAGs) and in particular heparan sulfate (HS) may play a role in the PrP(C) to PrP(Sc) conversion process. It has been proposed that PrP(Sc) accumulation in prion diseases may induce aberrant activation of lysosomal activity, which has been shown to result in neurodegeneration in a number of diseases, especially lysosomal storage disorders. Among such diseases, only the ones resulting from defects in GAGs degradation are accompanied by secretion of large amounts of GAG metabolites in urine. In this work, we show that GAGs are secreted in the urine of prion-infected animals and humans, and surprisingly, also in the urine of mice ablated for the PrP gene. We hypothesize that both the presence of PrP(Sc) or the absence of PrP(C) may alter the metabolism of GAGs.
journal_name
Neurobiol Disjournal_title
Neurobiology of diseaseauthors
Mayer-Sonnenfeld T,Zeigler M,Halimi M,Dayan Y,Herzog C,Lasmezas CI,Gabizon Rdoi
10.1016/j.nbd.2005.05.009keywords:
subject
Has Abstractpub_date
2005-12-01 00:00:00pages
738-43issue
3eissn
0969-9961issn
1095-953Xpii
S0969-9961(05)00144-0journal_volume
20pub_type
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