Therapeutic Approaches in Prion Disease

概要

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Prion protein (PrP) exists in two different isoforms ; a normal cellular isoform (PrP^C) and an abnormal infectious isoform (PrP^<Sc>), the latter is a causative agent of prion disease such as Bovine Spongiform Encephalopathy (BSE, mad cow disease) and Creutzfeldt-Jakob disease (CJD). Great concern about variant CJD, which is caused by ingesting BSE-contaminated products, is also emerging and spreading over Japan since the first BSE-affected cattle was identified in September, 2001. The amino acid sequences of PrP^C and PrP^<Sc> are identical, but their conformations are rather different ; PrP^C is rich in the non β-sheet isoform while PrP^<Sc> is rich in the β-sheet isoform. Our prion research focuses on further understanding such an unprecedented mechanism by identifying auxiliary factor (s) other than PrP^C and PrP^<Sc>. These studies also help us to develop "therapeutics and prevention methods" for prion disease. Three major trials ; genetic manipulation with dominant negative mutant PrP^C gene working against a hypothetical host-specific factor, antibody therapy with anti-PrP antibodies which block PrP^C-PrP^<Sc> binding, and PrP^<Sc> unfolding therapy with a novel-class molecular chaperone, are currently underway.
公益社団法人日本薬学会の論文
2003-08-01